Document:

EX-10.10

 Exhibit 10.10 
  

 
 Development and Clinical Supply Agreement 

Contract Number: E86A1D4C-B3F9 

Between 
 Rentschler
Biotechnologie GmbH 
 - and - 

Ultragenyx Pharmaceutical Inc. 

  
 [***] Certain information in this
document has been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

 CONTENTS 
  

							
	1	 	 Definitions
	  	 	3	  
			
	2	 	 Provision of Services
	  	 	6	  
			
	3	 	 Material and Technology Transfer
	  	 	9	  
			
	4	 	 Deliverables; Supply Failure; Acceptance & Rejection
	  	 	11	  
			
	5	 	 Quarantine Shipment and Quarantine Production
	  	 	12	  
			
	6	 	 Price and Payment Terms
	  	 	13	  
			
	7	 	 Steering Committee
	  	 	14	  
			
	8	 	 Audits and Inspections
	  	 	16	  
			
	9	 	 Warranties (“Gewaehrleistungen”)
	  	 	17	  
			
	10	 	 Liability and Indemnifications
	  	 	19	  
			
	11	 	 Confidential Information
	  	 	23	  
			
	12	 	 Intellectual Property Rights
	  	 	25	  
			
	13	 	 Term and Termination
	  	 	26	  
			
	14	 	 Force Majeure
	  	 	28	  
			
	15	 	 Period for Limitation of Ultragenyx Claims
	  	 	29	  
			
	16	 	 Applicable Law and Dispute Resolution
	  	 	29	  
			
	17	 	 Quality Agreement
	  	 	29	  
			
	18	 	 Miscellaneous
	  	 	30	  

  

			
	SCHEDULE ONE	  	Description of Cell Line and Ultragenyx Materials
		
	SCHEDULE TWO	  	Offer No. A-2012/5-00171, including description of Project, Initial Project Plan, Prices
		
	SCHEDULE THREE	  	Specifications (Schedule to be added during the course of the Project)
		
	SCHEDULE FOUR	  	Certificate of Insurance

  
 2 

 This Agreement (this “Agreement”) is made on the date of the last signature shown below
(“Effective Date”) 
 Between: 
  

	(1)	Rentschler Biotechnologie GmbH, Erwin-Rentschler-Straße 21, 88471 Laupheim, Germany (hereafter referred to as “Rentschler”); and, 

 

	(2)	Ultragenyx Pharmaceutical Inc., 60 Leveroni Court, Novato, CA 94949, USA (hereafter referred to as “Ultragenyx”). 

Purpose and Scope: 
  

	(A)	Rentschler is in the business of providing biotechnology development services including without limitation process development, validation, scale up services, production and product manufacturing services, quality
assurance, regulatory support, analytical development, fill and finish services and quality control analysis in respect of intermediate drug products and bulk form active pharmaceutical ingredients; and 

 

	(B)	Ultragenyx is in the business of drug development and wishes to engage and contract with Rentschler for development services pursuant to which Rentschler is to further develop Ultragenyx’s laboratory scale process
used in the manufacture of the recombinant enzyme beta-glucuronidase known as UX003 for initial scale up and validation of the process (“Project”). The Project is more fully described in the initial Project Plan in Schedule
Two; and, 

  

	(C)	Rentschler is willing to provide the Services pursuant to the Project to Ultragenyx which Ultragenyx is willing to accept on the terms and conditions set out in this Agreement. This Agreement relates to the Quality
Agreement with the contract number: 0467A7F4-15C7-. 

 Now it is Agreed as follows: 

 

	1	Definitions 

 For purposes of this Agreement, the terms defined in this Section shall
have the respective meanings set forth below: 
  

	 	1.1	“Affiliate” means any Company, partnership or other entity which directly or indirectly Controls, is controlled by or is under common control with the relevant Party to this Agreement.
“Control” means the ownership of more than fifty percent (50%) of the issued share capital or the legal power to direct or cause the direction of the general management and policies of the Party in question; 

 

	 	1.2	“Agreement” means this Development and Clinical Supply Agreement in its entirety including all Schedules; 

  
 3 

	 	1.3	“Applicable Laws” means all applicable laws, rules, regulations and guidelines that apply in Germany to the Services; 

  

	 	1.4	“Binding Reservation” means a reservation made by Ultragenyx to Rentschler to use the Rentschler facility for GMP manufacturing of Drug Substance, which reservation start date shall be agreed and documented in
writing by the Parties. 

  

	 	1.5	“Business Day” means any day which is not a Saturday, a Sunday or a German public holiday and references to any time shall be to German time (GMT+one hour); 

 

	 	1.6	“Cell Line” means the cell line as described in Schedule One or any other cell line agreed between the Parties in accordance with Section 3.2 below. Where a strain has been specified in the
Specification, references in this Agreement to Cell Line refer to that specified strain; 

  

	 	1.7	“Drug Substance” means the recombinant enzyme beta-glucuronidase; 

  

	 	1.8	“Drug Product” means the finished dosage form of the Drug Substance; 

  

	 	1.9	“Gewaehrleistungen” means certain contractual obligations hereunder, which in case of a breach have the consequences as ascribed to them in this Agreement, provided that in case of damages such damages must be
caused by negligence or willful misconduct; 

  

	 	1.10	“GMP” means Good Manufacturing Practices as regulated under the German Pharmaceuticals Act (“Arzneimittelgesetz”), the German Regulation for Manufacturing of Medicinal Products and Active Ingredients
(Arzneimittel- und Wirkstoffherstellungsverordnung), and the Guidelines to Good Manufacturing Practice of Medicinal Products for Human and Veterinary Use of the European Union, each of the foregoing as amended from time to time. Additionally, it
shall mean the compliance with the Code of Federal Regulations, Title 21, Chapter I, Parts 210 and 211, as amended from time to time; 

  

	 	1.11	“Change Order” has the meaning set forth in Section 2.7; 

  

	 	1.12	“Confidential Information” means any and all information of whatever nature relating to either Party and their business affairs and all such other information relating to technology of whatever nature and
application including, without limitation, all information relating to the Cell Line, Process, Product and strain, regardless of whether such information is specifically designated as confidential and regardless of whether such information is in
written, oral, electronic or other form; 

  

	 	1.13	“Group” means the relevant Party and Affiliates from time to time; 

  
 4 

	 	1.14	“Intellectual Property Rights” means all intellectual property rights, including (without limitation) patents, supplementary protection certificates, petty patents, utility models, trade marks, database
rights, rights in designs, copyrights and topography rights (whether or not any of these rights are registered, and including applications and the right to apply for registration of any such rights) and all inventions, know-how, trade secrets,
techniques and confidential information and other proprietary knowledge and information, and all rights and forms of protection of a similar nature or having equivalent or similar effect to any of these which may subsist anywhere in the world, in
each case for their full term, and together with any renewals or extensions; 

  

	 	1.15	“Party” shall mean Rentschler or Ultragenyx individually and “Parties” shall mean Rentschler and Ultragenyx jointly; 

 

	 	1.16	“Permitted Recipients” shall mean the employees of Rentschler, Inc., (the sales and distribution unit of Rentschler located at 400 Oser Ave,, Suite 1650, Hauppauge, NY 11788, USA) and the directors,
officers or employees of the respective Party who are required, on a strict need to know basis, in the course of their duties to receive and consider the Confidential Information for the purpose of enabling the relevant Party to perform its
obligations under this Agreement provided that such persons are under obligations of confidence no less onerous than those contained herein at Section 11 which are imposed on the recipient Party; 

 

	 	1.17	“Price” means the price for the Services as defined in Schedule Two; 

  

	 	1.18	“Process” means the method of manufacture and processing of the Product from the Cell Line which, pursuant to the Services, is to be further developed by Rentschler under this Agreement;

  

	 	1.19	“Product” means Drug Substance or Drug Product as required by the context; 

  

	 	1.20	“Project Plan” means the plan describing all activities to be performed under this Agreement, including any update and amendment of the Project Plan, as evidenced by a Change Order. The initial Project
Plan is included in Schedule Two; 

  

	 	1.21	“Quarantine Production” means any production of Product with materials that were not released or were delivered without a certificate of release; 

 

	 	1.22	“Quarantine Shipment” means any transport of a batch of Product before a certificate of release has been issued; 

  

	 	1.23	“Schedule” means the schedule (or schedules as appropriate) to this Agreement which specify the Services, payment terms and other relevant details referred to under this Agreement; 

 

	 	1.24	“Services” means any or all parts of the services to be performed under this Agreement in pursuance of the Project as more fully described in Schedule Two; 

  
 5 

	 	1.25	“Specifications” means the specifications of the Product as described in Schedule Three, as amended from time to time; 

 

	 	1.26	“Steering Committee” means the body organised in accordance with and pursuant to the rules under Section 7; 

  

	 	1.27	“Testing Laboratories” means any third party qualified according to Rentschler’s standard operating procedures that is instructed by Rentschler to carry out tests on the Cell Line, the Process,
Ultragenyx Materials or the Product pursuant to the performance of the Services under this Agreement; 

  

	 	1.28	“Ultragenyx Materials” means the Cell Line and all other materials provided by Ultragenyx, its Affiliates or agents to Rentschler and as described in Schedule One; 

 

	 	1.29	“Working Package” means a particular activity or series of activities that constitute a main step in the performance of the Services and which is more clearly identified in Schedule Two by the
breakdown of the Services into separate stages; 

  

	2	Provision of Services 

  

	 	2.1	Rentschler will perform the Services diligently using its professional skill and care in accordance with the terms of this Agreement and the Project Plan and will use its commercially reasonable endeavours to
meet the Specifications. Rentschler shall perform the Services under this Agreement according to the Project Plan, in accordance with GMP (as applicable per Section 2.2), and subject to terms and conditions as set out herein. Rentschler
will conduct all of its activities under this Agreement in compliance with all Applicable Laws. 

  

	 	2.2	Only those parts of the Services will comply with GMP that are explicitly designated to comply with GMP in Schedule Two. Should during the term of this Agreement new interpretations of GMP (the
“Interpretations”) become available, the Parties will discuss the Interpretations and decide on their application regarding the Services. Should the Parties be unable to reach an agreement on the applicability of Interpretations,
the final decision shall be with Ultragenyx. However, Rentschler is in any such case entitled to increase the Price if and to the extent Interpretations require additional Services directed specifically to the Process and/or Product (i.e., not to
include services which Rentschler would have to perform to be able to provide GMP conforming services also to other customers). 

  

	 	2.3	 As of the Effective Date, Ultragenyx is committed to engaging Rentschler to provide the Services and to paying Rentschler for such Services, in
each case, as generally described in Schedule Two. However, because of the inherent uncertainty in timelines for Product development, the Parties hereby agree to discuss and finalize, after the Effective Date, a Binding Reservation for
Rentschler’s GMP manufacturing of Drug Substance. Once a Binding 

  
 6 

	 	
Reservation has been made, but subject to the last paragraph in this Section 2.3, if Ultragenyx cancels the Binding Reservation, the Exit Fee is as follows: 

 

	 	2.3.1	In case of a cancellation during the period from and including the [***] to and including the [***] month before the start date of the Binding Reservation [***]% of the total remuneration for the respective
Services; 

  

	 	2.3.2	In case of a cancellation during the period from and including the [***] to and including the [***] month before the start date of the Binding Reservation [***]% of the total remuneration for the respective
Services; 

  

	 	2.3.3	In case of a cancellation during the period from the [***] to and including the [***] month before the start date of the Binding Reservation [***]% of the total remuneration for the respective Services;

  

	 	2.3.4	In case of a cancellation within [***] month before the start date of the Binding Reservation [***]% of the total remuneration for the respective Services. 

Rentschler shall use commercially reasonable efforts to mitigate any losses or damages it may incur due to Ultragenyx’s cancellation of
the Binding Reservation. If Rentschler mitigates such losses or damages so that it incurs no damage or significantly less damage as a result of the cancellation, Ultragenyx must only reimburse Rentschler for the unmitigated costs of the
cancellation. This applies in particular if Rentschler can use the production time for another comparable manufacturing. 
 For clarity, if
(1) Ultragenyx cancels the Binding Reservation because [***], then in either case, the foregoing Exit Fee [***]. 
  

	 	2.4	If (1) Ultragenyx cancels or postpones the Services concerning Drug Product for a reason within Ultragenyx’s control and not the result of Rentschler’s policies, or (2) other deviations to the
respective timelines stated in Schedule Two are caused by reasons within Ultragenyx’s control and not the result of Rentschler’s policies, then in either case, Rentschler is entitled to require that Ultragenyx pay the following
reimbursement for the unused production facilities: 

  

	 	2.4.1	In case of a cancellation, postponement or delay during the period from the [***] to and including the [***] week before the production time: [***]% of the remuneration for the respective Services;

  

	 	2.4.2	In case of a cancellation, postponement or delay during the period from the [***] week to and including the [***] week before the production time: [***]% of the remuneration for the respective Services;

  

	 	2.4.3	In case of a cancellation, postponement or delay during the period from the [***] week to and including the [***] week before the production time: [***]% of the remuneration for the respective Services.

  
 [***] Certain information in this document has been omitted and
filed separately with the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

  
 7 

 Rentschler shall use commercially reasonable efforts to mitigate any losses or damages it may
incur due to Ultragenyx’s cancellation, postponement or delay regarding the Drug Product. If Rentschler mitigates such losses or damages so that it incurs no damage or significantly less damage as a result of the cancellation, postponement or
delay in the provision of materials or information, Ultragenyx must only reimburse Rentschler for the unmitigated costs of the cancellation, postponement or delay. This in particular applies if Rentschler can use the production time for another
order. 
 For clarity, if Ultragenyx notifies Rentschler of a cancellation, postponement or delay prior to the [***] week before the
production time, then the foregoing reimbursement shall [***]. 
  

	 	2.5	Rentschler shall make available to Ultragenyx within a reasonable period of time upon completion of any Working Package as outlined in the Project Plan and – irrespective of the status of the Services –
upon reasonable request of Ultragenyx, copies of all relevant documentation and information resulting from the Services in order to enable Ultragenyx to evaluate the status and to meet any statutory or regulatory requirements. 

 

	 	2.6	In the performance of the Services Rentschler may subcontract certain part(s) of the Services to a Testing Laboratory and is permitted to sublicense to such Testing Laboratory, but only for the purpose of
performing those part(s) of the Services, any license from or grant of rights by Ultragenyx to Rentschler in respect of Ultragenyx Intellectual Property Rights and Ultragenyx Materials. Rentschler shall procure that all Testing Laboratories are
subject to confidentiality obligations and intellectual property obligations corresponding to the obligations under Section 11 and Section 12 below. For clarity, Rentschler shall obtain Ultragenyx’s prior written consent
before subcontracting Services to any third party that is not a Testing Laboratory. 

  

	 	2.7	The Services (including the Price thereof) as described on the Project Plan may be amended by a written change order that specifies the particular change(s), including any changes to the nature of the Services,
the timeline for such Services, and the Price associated with such Services (a “Change Order”). A Change Order may be submitted by either Party to the other, provided that such Change Order shall not be effective until signed by
Ultragenyx and Rentschler in accordance with the provisions of Section 18.1. 

  

	 	2.8	Ultragenyx acknowledges and accepts that the Services are dependent upon living systems and that they are experimental in nature. Accordingly, the Parties agree that because of these factors and the unpredictable
nature of the Services, the timelines and anticipated results expected by the Parties that are more fully described in the Schedules are estimates only and that provided Rentschler has complied with Section 2.1 it shall not be liable for
failing to achieve any target results, milestones or timelines or completing the Services. 

  

[***] Certain information in this document has been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has
been requested with respect to the omitted portions. 

  
 8 

	 	2.9	As Ultragenyx wishes to have a prompt start with the clinical program, Rentschler will manufacture the first batch of Drug Substance after the consolidation run according to GMP (“First Batch”).
Ultragenyx is aware that a successful GMP engineering run is required to confirm feasibility of the manufacturing of Drug Substance. Therefore, the Parties agree that Rentschler shall not be obligated to meet any Specifications for the First Batch
and Ultragenyx assumes all risks, responsibilities and costs associated with the manufacturing of the First Batch, except in case of damages caused by Rentschler’s gross negligence or willful misconduct. For the avoidance of doubt,
manufacturing as used in this Section shall also comprise the testing of the Product. The provisions in this section also apply to the first batch of Drug Product manufactured by Rentschler for Ultragenyx as, upon request by Ultragenyx, there will
not be a technical fill to confirm feasibility of the GMP-conform manufacturing of Drug Product. 

  

	 	2.10	As of the Effective Date, the Parties intend that Rentschler shall be the First Supplier to Ultragenyx of Product to the extent Ultragenyx demands such Product and Rentschler has available capacity and the
manufacturing is reasonably feasible at Rentschler’s facility. In such case, the Parties intend that “First Supplier” means that Ultragenyx will not source larger quantities of Product from another supplier than from Rentschler
per year. The Parties shall endeavor in good faith to negotiate a definitive agreement for commercial supply of Product in a timely manner. To this end, the Parties shall initiate negotiations, if possible before the planned production start for the
commercial supply, however, not later than the first submission of a BLA (US) or MAA (EU) for the Drug Product. For clarity, this Section 2.10 describes the Parties’ good faith intentions as of the Effective Date but shall not be
interpreted as a binding commitment on either Party with respect to Rentschler being a First Supplier to Ultragenyx nor the Parties concluding a definitive commercial supply agreement. 

 

	3	Material and Technology Transfer 

  

	 	3.1	Ultragenyx shall reasonably and timely support Rentschler as required to perform and to facilitate the proper provision of the Services and Ultragenyx shall deliver to Rentschler, DDP Rentschler’s facility
at Laupheim (Incoterms 2010), as of the Effective Date all Ultragenyx Materials and such other physical materials that are under Ultragenyx’s custody, control, ownership or influence that are necessary for Rentschler’s performance of the
Services including without limitation those materials identified in Schedule One. Ultragenyx shall at its own cost upon reasonable notice deliver to Rentschler further samples of such materials as are reasonably necessary and requested by
Rentschler during the Term of this Agreement to facilitate Rentschler’s proper performance of the Services. Ultragenyx shall inform Rentschler’s incoming goods department (ware- neingang@rentschler.de) and the project manager of any
delivery to be made according to this Section 3.1 at least [***] Business Days before any such delivery to Rentschler is triggered. Rentschler acknowledges that the Ultragenyx Materials may have biological properties that are
unpredictable and unknown at the time of transfer. However, Ultragenyx confirms that it has provided all safety 

  

[***] Certain information in this document has been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has
been requested with respect to the omitted portions. 

  
 9 

	 	
data and information available to Ultragenyx that are responsive to Rentschler’s safety requirements pertaining to the Cell Line and the Ultragenyx Materials, as set forth on Schedule
One. Ultragenyx represents and warrants that, to Ultragenyx’s knowledge, the Ultragenyx Material will not be contaminated and will not have hazardous properties except as disclosed in such safety data and information from Ultragenyx,
provided that Ultragenyx has tested the material in accordance with the corresponding provisions in Schedule Two. Additionally, Ultragenyx will provide Rentschler with any further data coming to Ultragenyx’s knowledge regarding the
safety of the Ultragenyx Materials. 

  

	 	3.2	Immediately upon supply of the Ultragenyx Materials to Rentschler, Rentschler shall inspect the Ultragenyx Materials in accordance with the Project Plan in order to determine whether the Ultragenyx Materials may
be acceptable and presumably suited for the Process according to the criteria listed in Schedule Two. Except for Rentschler’s negligent or willful breach of this Agreement, Rentschler shall not be liable for whatever reason for the
unsuitability of the Ultragenyx Materials for the Process. If Rentschler determines that it will not be able to use the supplied Ultragenyx Materials for the Process in accordance with this Agreement, it shall inform Ultragenyx hereof without undue
delay. In such event, Ultragenyx shall use all reasonable efforts to provide Rentschler with alternate, suitable Ultragenyx Material, and Rentschler shall use all reasonable efforts and will collaborate with Ultragenyx in order to select such other
cell line or other material, as the case may be, or make such reasonable modifications and adjustments to procure alternate Ultragenyx Material as soon as possible. If Rentschler refuses the initial Cell Line and Ultragenyx is unable to provide an
alternate, suitable Cell Line for the Process within a [***] week period, Ultragenyx shall have the right to terminate this Agreement in accordance with Section 13.3 below. In the event of any delays caused by an unsuitable Ultragenyx
Material and/or delayed receipt of Ultragenyx Material, Ultragenyx shall be liable for and shall bear any costs and consequences associated therewith and the timelines of the Project Plan shall be adjusted accordingly. 

 

	 	3.3	Ultragenyx shall make available to Rentschler as of the Effective Date in a useable and understandable format all Ultragenyx know-how and Confidential Information which is relevant to the Cell Line, Process,
Product, Services and the Project, including without limitation full and complete details of Ultragenyx’s laboratory scale process, that are necessary for Rentschler’s performance of the Services. 

 

	 	3.4	When reasonably required by Rentschler or where stipulated in any Schedule, Ultragenyx shall, at its own cost and expense, supply to Rentschler suitably skilled, educated and technical employees or
representatives with knowledge of the Project, Process, Cell Line, Product and Ultragenyx Materials for the purpose of facilitating and assisting the technology transfer of the Project technology to Rentschler to enable Rentschler to perform the
Services hereunder. Ultragenyx shall ensure that such employees or representatives will (i) be subject to enforceable obligations of confidentiality preventing them from using any information of a confidential nature which they acquire during
such visit; and (ii) obey the rules at Rentschler’s facility at Laupheim with regard to health and safety, GMP and Ultragenyx confidentiality. 

  

[***] Certain information in this document has been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has
been requested with respect to the omitted portions. 

  
 10 

	 	3.5	Ultragenyx hereby grants to Rentschler, for the Term of this Agreement and for the sole purpose of performing the Services in accordance with the Project Plan, a royalty free worldwide non-exclusive license in
respect of all Ultragenyx Intellectual Property Rights which are required by Rentschler for the proper performance of the Services hereunder. 

  

	 	3.6	Rentschler shall be responsible for the storage, handling and processing of the Ultragenyx Materials in accordance with Ultragenyx’s reasonable written instructions and the terms of the Quality Agreement.
Rentschler shall use the Ultragenyx Materials solely as necessary to conduct the Services and shall not sell, transfer, lease, or license the Ultragenyx Materials to any third party except as expressly permitted under Section 2.6.
Subject to Sections 10.5 through 10.7, Rentschler shall bear all risk of loss of the Ultragenyx Materials following delivery to Rentschler and shall replace at its cost any damaged or spoiled Ultragenyx Materials after delivery.

  

	4	Deliverables; Supply Failure; Acceptance & Rejection 

  

	 	4.1	Rentschler will throughout the performance of the Services keep Ultragenyx reasonably and thoroughly informed of the progress of the Services. 

 

	 	4.2	Upon conclusion of the Services and upon receipt of full payment in respect of sums properly owed to Rentschler hereunder, Rentschler shall deliver to Ultragenyx a comprehensive documentation on the Services
which shall comprise the results and reports generated pursuant to the Services. 

  

	 	4.3	Subject to Section 2.2, Rentschler will manufacture the Products for the use in clinical trials in accordance with GMP and the applicable Specifications according to Schedule Three and, if
requested by Ultragenyx, package and label the Products in accordance with applicable regulatory requirements and subject to conditions to be agreed between the Parties, and deliver the Products EXW Laupheim (as defined by Incoterms 2010) in
accordance with the estimated timelines in the Project Plan. Jointly with the delivery of the Products, Rentschler shall deliver to Ultragenyx the certificate of analysis, the certificate of conformity and such other documentation as is reasonably
required to meet all applicable statutory and regulatory requirements. 

  

	 	4.4	If, following validation of the Product, Rentschler fails to deliver on a timely basis the full amount of Product ordered, Ultragenyx shall have the right to require Rentschler to cancel any part of such order
with respect to which delivery has failed, provided that Rentschler may first attempt to remedy the failure in accordance with Section 13.2.2. The aforementioned remedy shall be Ultragenyx’s sole remedy in case Rentschler
successfully remedies such failure in accordance with Section 13.2.2. Further, should either Party perceive that a shortfall in delivery of Product by Rentschler is likely to occur for any reason, the Parties shall discuss appropriate
steps to alleviate such a shortfall. 

  
 11 

	 	4.5	Following validation of the Product, Ultragenyx may reject any portion of any shipment of Product that does not conform to the Specifications. In order to reject delivery of an order of Product, Ultragenyx must
give written notice to Rentschler of Ultragenyx’s rejection of any delivery within [***] days after receipt of such delivery, which notice shall specify Ultragenyx’s reason(s) for rejection. If no such notice of rejection is received
within [***] days of delivery of the order, Ultragenyx shall be deemed to have accepted such delivery of Product; provided, however, that in the case of Product having defects that are not discoverable upon reasonable physical inspection and testing
but are discovered at a later time (e.g., in the course or as a result of long-term stability studies) (“Latent Defect(s)”), Ultragenyx may reject such Product by giving written notice to Rentschler of Ultragenyx’s rejection of
such Product within [***] days after discovery of such Latent Defect(s). Product rejected by Ultragenyx will be returned to Rentschler at Rentschler’s request and at Rentschler’s expense. Whether or not Rentschler accepts Ultragenyx’s
basis for rejection, Rentschler will promptly initiate action to supply replacement Product at no additional cost after receiving Ultragenyx’s rejection notice. The aforementioned remedy shall be Ultragenyx’s sole remedy in case Rentschler
promptly initiates such corrective action and successfully supplies replacement Product within a reasonable time. Within [***] days of receiving any notice of rejection from Ultragenyx, Rentschler will respond stating whether (i) it accepts the
rejection or (ii) it disputes the rejection, in which case the Parties will refer such dispute to a mutually acceptable independent third party with the appropriate expertise to assess the conformity or non-conformity of the rejected Product to
Specifications. In the event the Parties are unable to agree within [***] calendar days on the person of such third party, each Party may request the appointment of such third party by the President of the Zurich Chamber of Commerce. The costs of
such expert opinion shall be borne equally by the Parties. Such independent third party shall test the applicable Product and shall determine whether such Product met or did not meet the applicable Specifications. The Parties agree that such third
party’s determination shall be final and binding upon the Parties. The Party against whom the independent third party rules shall bear the costs of testing by such independent third party. In case the independent third party determines that
Product did meet the Specifications, Ultragenyx will reimburse Rentschler for any actual costs incurred by Rentschler in providing such replacement of Product. 

  

	5	Quarantine Shipment and Quarantine Production 

  

	 	5.1	Rentschler may initiate a Quarantine Shipment upon Ultragenyx’s written request in order to provide that Ultragenyx receives the respective Products in a timely manner. 

 
 [***] Certain information in this document has been omitted and filed separately with
the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

  
 12 

	 	5.2	If Rentschler receives materials (especially active pharmaceutical ingredients) that are required for the performance of the Services and that were not released and/or were delivered without a complete
certificate of release, the project leader will inform Ultragenyx hereof promptly. Upon written request by Ultragenyx, Rentschler will conduct a preliminary analysis (identity, sterility and BSE/TSE) of the materials and perform a Quarantine
Production in order to ensure that the manufacturing process is not delayed. 

  

	 	5.3	Ultragenyx assumes all risks, responsibilities and costs associated with a Quarantine Shipment or Quarantine Production, except in case of Rentschler’s gross negligence or willful misconduct.

  

	6	Price and Payment Terms 

  

	 	6.1	Rentschler shall be paid the Price by Ultragenyx. The Price is exclusive of taxes, duties, levies, Value Added Tax, other fees of whatever nature imposed by or under the authority of any government or public
authority (other than taxes on Rentschler’s income), Capacity Fees or external costs (as indicated in Schedule Two), raw material costs or other expenses set out herein that Rentschler incurs or charges to provide the Services and which
Ultragenyx hereby agrees to pay. In respect of the external costs and raw material costs Rentschler has provided an estimate of those separate costs in Schedule Two but the final payment in respect of the same will be subject on the quantity
used and any changes in market price during the Term. An administrative and handling fee, not to exceed [***] percent ([***]%), will be added to costs accrued in connection with material supply, but not to other external costs (e.g. bulk harvest
safety testing, virus validation studies and analytics that are not performed by Rentschler). In each invoice, Rentschler shall itemize all external costs that exceed [***] Euros (€ [***]). 

 

	 	6.2	All prices are in Euro and all invoices will be raised in Euro and will be paid in Euro. 

  

	 	6.3	All payments shall be paid by Ultragenyx without any deductions or deferment and according to the payment schedule in Schedule Two. In the event that Ultragenyx disputes a portion of a particular
invoice, Ultragenyx shall so notify Rentschler within [***] days after receiving such invoice. The Parties shall thereafter discuss the disputed portion of the invoice. If the disputed portion of the invoice is less than [***] percent ([***]%) of
the total invoiced amount, then Ultragenyx shall pay the full invoiced amount and, if the Parties agree after discussion that such disputed amount was not properly owed to Rentschler, Rentschler shall credit Ultragenyx such disputed amount on the
following invoice or if no following invoice is expected, shall promptly reimburse Ultragenyx the disputed amount. If the disputed portion of the invoice is equal to or greater than [***] percent ([***]%) of the total invoiced amount, then
Ultragenyx shall not pay Rentschler such disputed portion of the invoice, but shall pay the undisputed portion, until the Parties have discussed and resolved the dispute. If the Parties agree after discussion that the disputed amount was properly
owed to Rentschler, 

  
 [***] Certain information in this document has
been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

  
 13 

	 	
Ultragenyx shall promptly pay Rentschler such amount. Ultragenyx shall have no right to retain undisputed sum(s) of such invoice and shall pay such sums in accordance with the terms of this
Section 6. 

  

	 	6.4	Rentschler’s hourly rates for the remuneration of Services as mentioned in this Agreement are: 

  

					
	 Analytical Work—Development
	  	€	[	***] 
	 USP/DSP Work
	  	€	[	***] 
	 Project Management
	  	€	[	***] 
	 Regulatory Affairs and Quality Assurance
	  	€	[	***] 
	 Parenterals Leading
	  	€	[	***] 
	 Analytical Work—Routine
	  	€	[	***] 
	 Parenterals Production
	  	€	[	***] 
	 Services (Technical Staff and the like)
	  	€	[	***] 

 Rentschler can adjust the hourly rates for the period of a further [***] months from the time of the start of
the [***] month onwards in the same ratio by which the [***] has changed. 
  

	 	6.5	In the event of default of payment of an undisputed sum on the due date, Rentschler shall give Ultragenyx no less than [***] days written notice following which if such payment has not been received in full,
Rentschler shall be entitled, at its own discretion and without any liability to Ultragenyx, to suspend the Services. 

  

	7	Steering Committee 

  

	 	7.1	The Parties shall—if not before—promptly after the Effective Date, form the Steering Committee which shall comprise a minimum of two (2) and an equal number of representatives
(“Representatives”) from each of Rentschler and Ultragenyx, and each Party shall notify the other of its elected Representatives. Each Representative shall carry an equal vote and proxy votes may be granted by Representatives to
their fellow Representative(s) if they are unable to attend meetings. The Steering Committee will take action by unanimous consent of its Representatives. 

  

	 	7.2	Each Party shall be entitled to change their respective nominated Representatives at any time and shall promptly give written notice of the change to the other Party including the new contact details of the new
Representative(s) in any event no less than [***] Business Days after the change has been implemented. 

  

	 	7.3	The quorum for the Steering Committee shall be a minimum of two (2) Representatives from each Party. The Steering Committee may meet in person or by telephone and advance written notice of not less than two
(2) Business Days must be communicated to no less than two (2) Representatives of each Party should a meeting be requested. 

  

[***] Certain information in this document has been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has
been requested with respect to the omitted portions. 

  
 14 

	 	7.4	The primary function of the Steering Committee is to ensure the ongoing communication between the Parties and discuss and resolve any issues arising under the Project. The Parties agree that their Representatives
will endeavour to attend each meeting and both the Representatives and each Party shall discuss events in good faith with the aim of furthering and successfully concluding the Project. In addition to the primary function described above the Steering
Committee shall also to take on the following responsibilities: 

  

	 	7.4.1	Discuss and seek resolution of issues around management of the Project; 

  

	 	7.4.2	Agree and monitor deadlines and milestones for the Project; 

  

	 	7.4.3	Agree and discuss amendments to the Specifications, including amendments due to comments or requirements from regulatory agencies, provided that in no event shall Rentschler implement any change to the
Specifications without Ultragenyx’s prior written consent and, in the event of a disagreement between Ultragenyx and Rentschler, and not withstanding anything to the contrary in Section 7.6, the Parties must jointly agree on changes
to the Specifications; 

  

	 	7.4.4	Agree and discuss any changes to the Services, provided that any change to the Services must be evidenced by a signed Change Order in accordance with Section 2.7. 

 

	 	7.5	The Steering Committee shall meet at such times as the Steering Committee determines reasonably necessary to monitor the progress of the Services and issues arising therefrom. Each Party may call for an
extraordinary meeting of the Steering Committee up to [***] times per calendar year with [***] day advance notice. 

  

	 	7.6	Should the Steering Committee be unable to reach agreement on any issue or should the Steering Committee not have met for a requested extraordinary meeting with the quorum according to Section 7.3
even after the second call for such a meeting, the issues shall be referred to a personal face-to-face meeting between senior executives of Rentschler and Ultragenyx both of whom shall act in good faith and discuss the issues to seek a resolution
amicably acceptable to both Parties and if resolved the resolution shall be binding and final. The meeting shall take place within [***] days of the date of the relevant referral. In case such persons cannot agree within further [***] days after
such face-to-face meeting, then the following shall apply: 

  

	 	7.6.1	If the dispute is predominantly concerned with a scientific or technical issue then the entire dispute shall be referred to an independent third party expert (appointed jointly by the Parties who is an expert in
the particular scientific or technical area at issue and who shall act as an expert and not an arbitrator). In the event the Parties are unable to agree within [***] calendar days on the person of such third party, each Party may request the

  
 [***] Certain information in this document has been omitted and
filed separately with the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

  
 15 

	 	
appointment of such third party by the President of the Zurich Chamber of Commerce. The costs of such expert opinion shall be borne equally by the Parties. The decision of the independent expert
shall be given in writing and in English and considered final and binding on the Parties except if there has been a manifest error on the face of the decision whereupon the Parties may revert to their respective remedies under Section 16.2.

  

	 	7.6.2	If the dispute is predominantly concerned with an issue other than scientific or technical then it shall be resolved in accordance with Section 16.2 below. 

 

	8	Audits and Inspections 

  

	 	8.1	Audits 

 Ultragenyx shall be entitled to an initial [***]-day audit with one auditor or
one team of Rentschler’s production site and related facilities used for the Services and to a [***]-day audit of one auditor or one team per year with [***] months prior notice. Ultragenyx will have the option, at Ultragenyx’s expense, to
designate an independent third party, reasonably acceptable to Rentschler, to verify that all Services according to Section 2.2 are in compliance with GMP and all Applicable Laws. Further, Ultragenyx may audit those parts of
Rentschler’s books and records which contain specific entries referring to Ultragenyx, if there is competent evidence that a material issue or a violation has occurred or will occur with respect to the U.S. Foreign Corrupt Practices Act of 1977
(as amended) (the “FCPA”) or the U.S. Travel Act. For the avoidance of doubt, this does not entitle Ultragenyx to pre-trial discoveries in the sense of US procedural law. Any additional audit will be charged to Ultragenyx on a time
and material basis depending on the scope of such audit. Notwithstanding the foregoing, if any such audit is a for-cause audit or follow up audit of an audit during which deficiencies or conditions requiring corrective action were identified, then
the notice period shall be reduced to thirty days and Rentschler will not charge Ultragenyx for any costs or expenses (whether internal or external) related to such audit. 
  

	 	8.2	Inspections 

 In respect of GMP Services, Rentschler shall reasonably permit a
governmental or regulatory authority body to enter those areas of Rentschler’s premises concerned with the Services for the sole purpose of observing and inspecting the performance of the GMP Services and those records of Rentschler specific to
the GMP Services where such entry is reasonably necessary or mandatory in order for Ultragenyx to maintain, approve, apply for or amend its regulatory application or approval in respect of the Product. Such inspections are subject to (i) the
individuals representing such government or regulatory authority body obeying and adhering to the rules and regulations in place at Rentschler concerning health and safety, GMP and Ultragenyx confidentiality; and (ii) in the case of 

 
 [***] Certain information in this document has been omitted and filed separately with
the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

  
 16 

 
inspections by a governmental or regulatory authority body other than the regional council Tübingen (“Regierungspräsidium Tübingen”), Rentschler being entitled to charge
Ultragenyx for such inspections at Rentschler’s then standard rates, provided that such charges shall not exceed [***] Euros (€ [***]) for an audit consisting of [***] auditors for [***] days duration. 

 

	9	Warranties (“Gewaehrleistungen”) 

  

	 	9.1	Ultragenyx warrants (“gewährleistet”) to Rentschler that: 

  

	 	9.1.1	it is legally incorporated and in good standing in its country of incorporation and that it has the right to enter into this Agreement. 

 

	 	9.1.2	it has the right to supply or license Rentschler with Ultragenyx Materials, Ultragenyx Intellectual Property Rights, and Confidential Information and, if necessary, has obtained for the benefit of Rentschler and
advised Rentschler accordingly of all necessary license(s) as known to Ultragenyx’ best knowledge as of the Effective Date to allow Rentschler to use the transferred Process, Ultragenyx Materials, Ultragenyx Intellectual Property and
Confidential Information for the provision of the Services, development of the Process and manufacture of Product; 

  

	 	9.1.3	to its best knowledge as of the Effective Date, the Ultragenyx Materials are free of all contaminants including without limitation any virus, bacteria (other than the Cell Line itself), prions, or other vectors
and will not pose a health hazard or biohazard; 

  

	 	9.1.4	where it has provided Rentschler with or directed or stipulated that Rentschler use the Cell Line, to Ultragenyx’s best knowledge as of the Effective Date, Rentschler is lawfully entitled to use the Cell
Line for the provision of the Services and manufacture of Product; 

  

	 	9.1.5	to its best knowledge as of the Effective Date, the use of the Cell Line, Ultragenyx know-how, Confidential Information, Ultragenyx Intellectual Property Rights and Ultragenyx Materials by Rentschler for the
development of the Process and the manufacture of Products does not infringe any Intellectual Property Rights of a third party; 

  

	 	9.1.6	it will promptly inform Rentschler if it receives notice of any claim or potential claim relating to infringement or alleged infringement of any third party Intellectual Property Rights by virtue of
Ultragenyx’s or Rentschler’s use of the Cell Line, Ultragenyx Information or Ultragenyx Materials or the manufacture of Product; and 

  

	 	9.1.7	to its best knowledge as of the Effective Date, the Ultragenyx Materials, Ultragenyx Intellectual Property Rights and Confidential Information transferred to Rentschler are all that is necessary for Rentschler to
perform the Services (in addition to the Rentschler Intellectual Property Rights, Rentschler know-how and Rentschler’s Confidential Information) and are suitable for the performance of the Services; and 

 
 [***] Certain information in this document has been omitted and filed separately with
the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

  
 17 

	 	9.1.8	it shall not use, or make available for use, Product for human consumption except in human clinical trials in accordance with GCP. 

 

	 	9.2	Rentschler warrants (“gewährleistet”) to Ultragenyx that: 

  

	 	9.2.1	it is legally incorporated and in good standing in its country of incorporation and that it has the right to enter into this Agreement; 

 

	 	9.2.2	it has or will establish the necessary permits (including permit for work in a GMP environment), facilities and technically qualified employees that may be reasonably anticipated to be required for provision of
the Services; 

  

	 	9.2.3	it shall diligently perform the Services in accordance with its obligations under Section 2.1; 

  

	 	9.2.4	to its best knowledge, the Rentschler Intellectual Property Rights and Rentschler know-how do not, on their own, infringe any Intellectual Property Rights of a third party; 

 

	 	9.2.5	to its best knowledge, the Rentschler Intellectual Property Rights and Rentschler know-how are all that is necessary for Rentschler to perform the Services (in addition to the Ultragenyx Intellectual Property
Rights, Ultragenyx know-how and Ultragenyx’s Confidential Information) and are suitable for the performance of the Services; 

  

	 	9.2.6	it will not knowingly infringe or misuse a third party’s Intellectual Property Rights in its performance of the Services; 

 

	 	9.2.7	it will not misuse, sell or unlawfully disclose to a third party Ultragenyx Materials, Ultragenyx Intellectual Property Rights, Ultragenyx know-how and Confidential Information, nor assist another to do so;

  

	 	9.2.8	it will use its reasonable endeavours to keep the Ultragenyx Materials and Cell Line safe and secure; 

  

	 	9.2.9	it will convey unencumbered title (save for any intellectual property rights which may exist) to Product to Ultragenyx upon delivery; 

 

	 	9.2.10	it will promptly inform Ultragenyx if it receives notice of any claim or potential claim relating to infringement or alleged infringement of any third party Intellectual Property Rights by virtue of its use of
the Cell Line, Ultragenyx Information or Ultragenyx Materials or the manufacture of Product; 

  
 18 

	 	9.2.11	it will not with a corrupt intent directly or indirectly offer, promise, authorize, pay or give any money, favor, advantage, bribe, kickback, or anything else of value to any government official or entity or to
any other individual or entity for purposes of obtaining, retaining, or directing business or any other improper advantage; 

  

	 	9.2.12	it has in good faith provided to Ultragenyx all documents and information of the character and type requested by Ultragenyx in writing in the course of Ultragenyx’s due diligence review of Rentschler. To
Rentschler’s best knowledge, there are no documents or information of a character or type described in such request which have not been so provided by Rentschler; and 

 

	 	9.2.13	it shall respond to Ultragenyx’s requests for information, to the extent reasonable and related to Ultragenyx’s efforts to ensure compliance with the FCPA and any other applicable anti-corruption law.

  

	 	9.3	In consideration of the express warranties set out above under this Section 9, to the maximum extent permitted by Applicable Laws and Swiss law (save for those express warranties set out above)
neither Party makes or gives any other express or implied (whether by statute, custom or otherwise) warranties in relation to its respective obligations, duties or activities owed or performed under this Agreement and hereby excludes all such
warranties. 

  

	 	9.4	If Ultragenyx finds any Service not to meet the warranties given by Rentschler in accordance with Section 9.2.3, Ultragenyx may upon notice to Rentschler require Rentschler to provide Services, which
fully satisfy the given warranties of Section 9.2.3 at Rentschler’s sole risk and expense within the time periods stated in Section 13.2.2 for curing the breach. In the event, however, such remedial Services do not meet
the warranties of Section 9.2.3 and Rentschler fails to rectify Ultragenyx’ warranty claim within the time periods stated in Section 13.2.2, Ultragenyx may terminate this Agreement in accordance with
Section 13.2.2, and/or claim damages and indemnification in accordance with Section 10, including the limitation of liability provisions stated in Sections 10.5 through 10.7. 

 

	10	Liability and Indemnifications 

 Indemnification 

 

	 	10.1	Ultragenyx shall indemnify and hold harmless Rentschler and each of its directors, officers, employees, shareholders, and Testing Laboratories (the “Rentschler Parties”) against any and all
losses, demands, claims, liabilities, damages, costs and expenses (including but not limited to consequential losses and loss of profit, court costs and documented attorney’s fees and expenses together with any applicable taxes thereon) brought
or incurred by third parties to the extent resulting from the following: 

  

	 	10.1.1	any material unremedied breach by the Ultragenyx Parties of the warranties given pursuant to Section 9.1; 

  
 19 

	 	10.1.2	any infringement or alleged infringement or breach of any third party rights by any Party, including without limitation any intellectual or industrial property rights, patents, trademarks, copyright, know-how or
confidential information, by use of the Ultragenyx Intellectual Property Rights and/or Ultragenyx know-how and/or Ultragenyx Confidential Information and/or Ultragenyx Materials in the performance of the Services; 

 

	 	10.1.3	any product liability claims relating to the Product or Ultragenyx Materials including any derivatives of the foregoing, conjugated form or formulation of the same to the extent such claim is based on the use of
the Product following the manufacturer’s release (except where such liability has arisen due solely to an act of at least gross negligence caused by Rentschler and is not covered under Ultragenyx’s product liability insurance);

  

	 	10.1.4	the negligence of Ultragenyx in relation to the use, processing, storage or sale of the Product or any derivative, conjugated form or formulation thereof; or 

 

	 	10.1.5	any negligence or willful misconduct by Ultragenyx designed and intended to cause detriment to Rentschler in performance of its obligations under this Agreement; 

provided, however, that Ultragenyx shall have no obligation to indemnify Rentschler to the extent that any losses, demands, claims liabilities,
damages, costs and expenses are caused by Rentschler’s own negligence or willful misconduct in the performance of the Services or by Rentschler’s at least negligent breach of this Agreement. 

 

	 	10.2	Rentschler shall indemnify and hold harmless Ultragenyx and each of its directors, officers and employees (the “Ultragenyx Parties”) against any and all losses, demands, claims, liabilities,
damages, costs and expenses (including but not limited to reasonable court costs and documented attorney’s fees and expenses together with any applicable taxes thereon) brought or incurred by third parties to the extent resulting from the
following: 

  

	 	10.2.1	any material unremedied breach by the Rentschler Parties of the warranties given pursuant to Section 9.2; 

  

	 	10.2.2	any infringement or alleged infringement or breach of any third party rights by any Party, including without limitation any intellectual property rights, patents, trademarks, copyrights, know-how or confidential
information, by use of the Rentschler Intellectual Property Rights and/or Rentschler know-how in the performance of the Services; 

  
 20 

	 	10.2.3	the negligence of Rentschler in relation to the use, processing, storage or sale of the Product or any derivative, conjugated form or formulation thereof; or 

 

	 	10.2.4	any negligence or willful misconduct by Rentschler designed and intended to cause detriment to Ultragenyx in performance of its obligations under this Agreement; 

provided, however, that Rentschler shall have no obligation to indemnify Ultragenyx to the extent that any losses, demands, claims liabilities,
damages, costs and expenses are caused by Ultragenyx’s own negligence or willful misconduct in the performance of its rights obligations hereunder or by Ultragenyx’s at least negligent breach of this Agreement. 

Indemnification Procedure 
  

	 	10.3	The Party (the “Indemnitee”) that intends to claim indemnification under this Section 10 shall: 

  

	 	10.3.1	promptly, and in any event within [***] Business Days of it receiving notice of the claim, demand, threat or action, notify the other Party (the “Indemnitor”) in writing in general terms of any
claim, demand, threat or action which has or has the potential to give rise to the Indemnitee seeking to rely on and claim the benefit of the indemnification together with notification of the Indemnitee’s intention to rely on such indemnity,
provided however, that failure to give such notice shall not relieve the Indemnitor of its indemnification obligations except and only to the extent such failure actually and materially prejudices the ability of the Indemnitor to defend against such
claims; 

  

	 	10.3.2	not prejudice any defense to any claim or attempt to settle or compromise such claim; 

  

	 	10.3.3	subject to its other rights and obligations and compliance with the procedures set out in this Section 10 permit the Indemnitor to have overall control of the conduct of the negotiations and the
proceedings including any counterclaim; 

  

	 	10.3.4	cooperate as reasonably requested by the Indemnitor, at the Indemnitor’s expense, in the conduct of such claim (and any counterclaim); and 

 

	 	10.3.5	have the right (at the Indemnitor’s expense) to instruct independent counsel and participate in all proceedings and negotiations whether named or not as a party in the claim or proceedings.

  

	 	10.4	The Indemnitor shall not settle or consent to an adverse judgment in any such claim, demand, action or other proceeding that adversely affects the rights or interests of any Indemnitee or imposes additional
obligations (financial or otherwise) on such Indemnitee, without the prior express written consent of such Indemnitee (such consent to be at the Indemnitee’s sole discretion). 

 
 [***] Certain information in this document has been omitted and filed separately with
the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

  
 21 

 Limitation of Liability 

 

	 	10.5	In no event shall either Party be liable to the other Party for incidental, indirect or consequential damages or loss of profit arising from or related to a breach of this Agreement. The foregoing disclaimer of
damages shall not apply in the case of (i) a breach of Section 11 (Confidentiality), (ii) personal injury or death, (iii) gross negligence or intentionally wrongful acts or omissions, or (iv) to third party claims
subject to the indemnification provisions in Section 10.1 and Section 10.2. 

  

	 	10.6	Rentschler’s aggregate liability to Ultragenyx for any loss or damage suffered by Ultragenyx as a result of Rentschler’s negligent or willful breach of this Agreement or of any other liability in
respect to Rentschler’s performance of the Services shall be except in cases of gross negligence or intentionally wrongful acts limited to 

  

	 	10.6.1	[***] percent ([***]%) of the [***] in case of negligence, or 

  

	 	10.6.2	the amount paid by Rentschler’s general liability insurance, whichever amount is higher. 

  

	 	10.7	The Parties’ liability and indemnification obligation as agreed to under this Section 10 shall be the Parties’ sole and exclusive liability and indemnification obligation towards each other
and any further liability and indemnification obligation shall be explicitly excluded to the extent permitted by applicable law. 

  

	 	10.8	Insurance 

 Prior to the first commercial sale of a Product Ultragenyx shall
obtain and shall thereafter maintain for [***] years after termination of this Agreement product liability insurance with a reputable and solvent insurance provider in an amount of at least $[***] per occurrence and in the aggregate. Such product
liability insurance shall insure against all mandatory liability including liability for personal injury, physical injury and property damages. Further, Ultragenyx shall obtain any necessary patients insurance required to perform the clinical
studies. Ultragenyx has obtained and will maintain during the Term of this Agreement general liability insurance of $[***] per occurrence and $[***] in the aggregate. Rentschler shall obtain and maintain during the Term of this Agreement and for
[***] years thereafter-general liability insurance with a reputable and solvent insurance provider. Reference is made to Rentschler’s current certificate of insurance attached hereto as Schedule Four. The Parties shall provide each other
written proof of the existence of such insurance upon request. 
  
 [***] Certain
information in this document has been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has been requested with respect to the omitted portions. 

  
 22 

	11	Confidential Information 

  

	 	11.1	The Parties acknowledge that there may be disclosure of each other’s Confidential Information to the other. In consideration of the disclosing Party making available its Confidential Information to the
recipient Party, the recipient Party undertakes that it shall, and shall procure that each of its Permitted Recipients, shall: 

  

	 	11.1.1	treat and safeguard as private and confidential all the Confidential Information; 

  

	 	11.1.2	use the Confidential Information only for those purposes reasonably required or anticipated under this Agreement and without prejudice to the generality of the foregoing, not use any Confidential Information to
obtain any commercial advantage over the disclosing Party; 

  

	 	11.1.3	ensure the proper and secure storage of all Confidential Information applying standards of care reasonably expected and no less stringent than standards applied to protection of recipient Party’s own
confidential information; 

  

	 	11.1.4	not at any time without the disclosing Party’s prior written consent disclose or reveal, whether directly or indirectly any of the Confidential Information to any person whatsoever save its Permitted
Recipients, and then on a limited need to know basis, who shall be informed by it of the confidential nature of the Confidential Information and of the confidentiality terms of this Agreement and for whom it hereby accepts full responsibility in the
event that any such person shall breach the duty of confidence imposed upon them; and 

  

	 	11.1.5	not at any time have any discussion, correspondence or contact with any third party concerning the Confidential Information without the prior written consent of the disclosing Party. 

 

	 	11.2	The obligations in this Agreement regarding Confidential Information do not apply to the extent that the recipient Party can prove by written evidence that the respective Confidential Information:

  

	 	11.2.1	is, at the time of its disclosure by the disclosing Party, wholly available to the public and could be obtained without reference to the Confidential Information by any person with no more than reasonable
diligence; 

  

	 	11.2.2	becomes generally available to the public after such disclosure otherwise than by reason of a breach of any of the undertakings in this Agreement or any breaches of confidence by the recipient Party or its
Permitted Recipients; 

  

	 	11.2.3	is, at the time of such disclosure, lawfully already within its possession; 

  
 23 

	 	11.2.4	is independently developed by the recipient Party without reference to the Confidential Information of the disclosing Party, as evidenced by records; or 

 

	 	11.2.5	was disclosed to an information technology service provider to implement, operate and/or maintain receiving Party’s technical or software internal systems, provided that the information technology service
provider is sworn to secrecy and confidentiality obligations at equal terms as stipulated in this Agreement. 

 In addition,
each Party may disclose Confidential Information of the other Party to the extent such disclosure is required by law or by any stock exchange or other regulatory authority having jurisdiction (but, for the avoidance of doubt, only to that extent).

  

	 	11.3	For the avoidance of doubt, no provision in this Agreement shall restrict each Party’s right to disclose the existence of a business relationship between the Parties to potential customers.

  

	 	11.4	Other than the limited and restricted rights of use set out in this Section 11 nothing in this Agreement intends to or has the effect of granting any right, title, license or interest in or to the
recipient Party in respect of the disclosing Party’s Confidential Information. 

  

	 	11.5	If the recipient Party or any of its Permitted Recipients becomes compelled to disclose any Confidential Information in the circumstances described in Section 11.2 of this Agreement or a breach or
threatened breach of this Section 11 occurs or becomes apparent or becomes aware of any misuse of the Confidential Information, the recipient Party shall inform the disclosing Party in writing of such obligation or fact as soon as
possible after it is informed, or becomes aware, of it and if possible, before any Confidential Information is disclosed, so that (if the disclosing Party in its absolute discretion shall see fit) a protective order or other appropriate remedy may
be sought. The recipient Party agrees to assist and co-operate (and shall procure that each of its Permitted Recipients shall, as appropriate, assist and co-operate) in any action which the disclosing Party may decide to take. The recipient Party
shall notify the disclosing Party prior to each disclosure of Confidential Information if it is under any obligation which would or might compel it to disclose any Confidential Information and subsequent to such disclosure it shall not voluntarily
assume any such obligation. 

  

	 	11.6	Except as otherwise provided for in this Agreement or otherwise required by law or administrative authorities, neither Ultragenyx nor Rentschler shall disclose any terms or conditions of this Agreement to any
third party without the prior written consent of the other Party. 

  
 24 

	 	11.7	Upon termination of this Agreement or at the request of the disclosing Party, each Party shall promptly return to the other, at the other’s request, any and all Confidential Information of the other
(including copies of documents, computer records and records on all other media) then in its possession or under its control except that the receiving Party may retain one (1) copy of all such Confidential Information for legal archival
purposes only. The receiving Party shall not be required to destroy any computer files stored securely by the receiving Party that are created during automatic system back-up. 

 

	 	11.8	The provisions of this Section 11 shall survive termination or expiry of this Agreement by the shorter of (i) [***] years after first commercial launch of a Product or (ii) [***] years after
termination or expiry of this Agreement. 

  

	 	11.9	For the purposes of this Section Confidential Information of Cellca GmbH shall be deemed to be Confidential Information of Rentschler. 

 

	12	Intellectual Property Rights 

  

	 	12.1	Rentschler and Ultragenyx agree that any manufacturing process, protocol, assay, and formulation developed in the course of Rentschler’s performance of its obligation under this Agreement (together with all
intellectual property rights therein) will be: (a) solely owned by Ultragenyx if solely related to Product, and Rentschler hereby assigns to Ultragenyx all of its rights and interest therein, provided that, in case of any invention made by an
employee of Rentschler (“Inventor”), Ultragenyx compensates Rentschler for any payments due to the Inventor according to the German Act on Employee Inventions (“Arbeitnehmererfindungsgesetz”); and (b) solely owned by
Rentschler if generally applicable to the manufacture of other products in addition to Product, and Rentschler hereby grants to Ultragenyx a fully-paid royalty-free, worldwide, perpetual license thereunder, with the right to grant sublicenses in
multiple tiers, for Ultragenyx to make, have made, use, sell, offer for sale and import Ultragenyx products, including Product and its derivatives and analogues. In addition, Rentschler hereby grants Ultragenyx a fully-paid, royalty-free, worldwide,
perpetual license, with the right to grant sublicenses in multiple tiers, under other patents and know-how owned or in-licensed by Rentschler that cover technology incorporated into the Services provided by Rentschler under this Agreement, for
Ultragenyx to make, have made, use, sell, offer for sale and import Ultragenyx products, including Product and its derivatives and analogues (“Rentschler’s Background IP”). Upon Ultragenyx’s request, Rentschler shall
transfer to Ultragenyx or its designee the know-how required for the exercise of the license granted to Ultragenyx hereunder regarding Rentschler’s Background IP. 

 

	 	12.2	Ultragenyx hereby grants to Rentschler a license under the Ultragenyx Intellectual Property Rights as specified in Section 3.5. Other than this license, Rentschler shall not acquire any rights
whatsoever in Ultragenyx Intellectual Property Rights and Rentschler acknowledges that these rights belong exclusively to Ultragenyx. 

  

[***] Certain information in this document has been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has
been requested with respect to the omitted portions. 

  
 25 

	 	12.3	Each Party may file patent protection on any Intellectual Property Rights it owns or, as set forth above, subsequently owns under this Agreement and the other Party shall promptly upon request co-operate, at the
requesting Party’s reasonable expense, with any requests to assist or enable the Party’s protection including but not limited to signing and delivering documents and other information necessary for the valid application and prosecution of
any such patent. 

  

	13	Term and Termination 

  

	 	13.1	This Agreement shall commence on and become effective on the Effective Date provided that it has been lawfully executed by all Parties and will expire when the Services have been completed or terminated in
accordance with this Section 13 (the “Term”). In respect of any part(s) of the Services which have been performed before the Effective Date the Parties agree that the performance of those part(s) shall be deemed to have
been performed during the Term and governed by the terms of this Agreement. 

 Events of Termination 

 

	 	13.2	Subject to force majeure and notwithstanding Section 13.1, either Party (“Non-Defaulting Party”) may terminate this Agreement before expiry of the Term with immediate effect upon
written notice to the other Party (“Defaulting Party”) if: 

  

	 	13.2.1	the Defaulting Party fails to pay any sum payable under this Agreement thirty (30) days after a written demand issued after the original due date; 

 

	 	13.2.2	the Defaulting Party makes or has made a material misrepresentation or commits a material breach of its obligations under this Agreement and, if the breach is capable of remedy, fails to remedy it during the
period of thirty (30) days starting on the date of receipt of notice from the Non-Defaulting Party generally identifying the breach and requiring it to be remedied. Notwithstanding the foregoing, in the event the alleged breach in question is
not reasonably capable of cure within the foregoing thirty (30) day period, but is otherwise capable of being cured within an additional one hundred fifty (150) days, the Defaulting Party may submit a reasonable cure plan prior to the end
of such initial thirty (30) day cure period, in which case, the Non-Defaulting Party shall not have the right to terminate under this Section 13.2.2 with respect to such alleged breach for so long as the Defaulting Party is
diligently implementing such cure plan, provided, however, that if the Defaulting Party fails to cure such breach (even if diligently implementing a cure plan) within one hundred eighty (180) days from the date of the first written notice
identifying the material breach in reasonable detail, then the Non-Defaulting Party may terminate under this Section 13.2.2 upon expiration of such one hundred eighty (180) day period. 

  
 26 

	 	13.2.3	(i) the Defaulting Party is deemed unable to pay its debts within the meaning of Sections 17, 18 or 19 of the German Insolvency Act, or (ii) the assets of the Defaulting Party became part of
insolvency proceedings, such right to terminate not existing if and as long as the (provisional) administrator continues business operations of the Defaulting Party and payments and counter payments are guaranteed for the time of administration, or
(iii) an application to open insolvency proceedings at the Defaulting Party has been dismissed due to lack of assets; or (iv) any shareholders’ meeting is convened for the dissolution of the Defaulting Party. 

 

	 	13.2.4	the Defaulting Party ceases to carry on its business for a period of no less than sixty (60) days; 

  

	 	13.2.5	the Defaulting Party suffers, or there occurs in relation to that Party, any event which in the reasonable opinion of the Non-Defaulting Party is analogous to any of the events mentioned in Sections 13.2.1
to 13.2.4; 

  

	 	13.3	Notwithstanding Section 13.1 Rentschler and Ultragenyx may terminate this Agreement or any part of the Services on thirty (30) days advance written notice if the Steering Committee concludes that
the Services cannot technically or scientifically or in any other way be delivered in accordance with this Agreement, and Ultragenyx may terminate this Agreement hereunder as set forth in Section 3.2 above. In the event this Agreement is
terminated in accordance with this Section 13.3, Ultragenyx shall pay to Rentschler the sums specified pursuant to Section 13.5. 

  

	 	13.4	Ultragenyx may terminate this Agreement at any time before completion of the Services by giving no less than sixty (60) days advance notice in writing to Rentschler. In the event Ultragenyx elects to
terminate this Agreement in accordance with this Section 13.4, it shall pay to Rentschler the sums specified pursuant to Section 13.5. 

Consequences of Termination 
  

	 	13.5	In the event that this Agreement is terminated pursuant to Section 13.2 by Rentschler or based on the other extraordinary termination rights by either Party, neither Party shall incur any future
liability towards the other Party other than: 

  

	 	13.5.1	in respect of any accrued rights; 

  

	 	13.5.2	the payment by Ultragenyx to Rentschler of sums due in respect to those Working Packages completed in accordance with this Agreement at the date of termination and a pro-rata Price for those Working Packages
(fairly determined having regard to man hours, materials, profit element and uncancellable commitments incurred and unmitigated by Rentschler; provided that Rentschler shall use commercially reasonable efforts to mitigate any costs it may incur)
which, at the date of termination, have not been completed but have been started and performed in accordance with this Agreement including all raw materials and other external costs incurred by Rentschler in performance of the Services; and,

  
 27 

	 	13.5.3	in the event this Agreement is terminated pursuant to Section 13.2 by Rentschler or this Agreement or individual Working Package(s) are terminated by Ultragenyx based on the other extraordinary
termination rights, Ultragenyx shall pay to Rentschler, in addition to any other sums payable under this Section 13.5, the amounts stated in Section 2.3 and Section 2.4 as applicable. For the avoidance of doubt,
any payments made under this Section 13.5 shall not exceed the total Price of the Services plus actual costs incurred for raw materials and other external costs. 

 

	 	13.6	Termination of this Agreement for whatever reason shall not affect the accrued rights of either Rentschler or Ultragenyx arising under or out of this Agreement and all provisions which are expressed to survive
this Agreement and the provisions of Sections 9, 10, 11, 12, 13, and 15 through 18 shall survive termination or expiry and remain in full force and effect. 

 

	 	13.7	The rights and licenses and the like granted in accordance with this Agreement and any claims either Party might have (whether or not notified to the other Party prior to the Termination) against the other Party
as well as the liability and insurance provision of this Agreement shall unless otherwise stated survive the termination of this Agreement. 

  

	 	13.8	Upon expiry of this Agreement or termination of this Agreement pursuant to Section 13.2, Section 13.3 or Section 13.4 by either Party, Rentschler shall without undue delay transfer to
Ultragenyx all data and information necessary to exercise the manufacture (e.g., cell banks, fermentation processes, product identity assays, in-process-control assays, standard operating procedures), make available Process related media and feeds,
and furnish all assistance and answer all questions received from Ultragenyx regarding the Process, in order to allow Ultragenyx or a third party designated by Ultragenyx to replicate the Process and take over the manufacturing of Products. The
costs of such transfer and assistance shall be borne by Ultragenyx. 

  

	 	13.9	For clarity, in the event this Agreement is terminated by Ultragenyx pursuant to Section 13.2, Ultragenyx shall not owe Rentschler the amounts stated in Section 2.3 and Section 2.4.

  

	14	Force Majeure 

  

	 	14.1	Neither Party shall be held liable or responsible to the other Party nor be deemed to have defaulted under or breached this Agreement for failure or delay in fulfilling or performing any term of this Agreement or
the Services to the extent, and for so long as, such failure or delay is caused by or results from causes beyond the reasonable control of such Party including but not limited to fires, earthquakes, floods, embargoes, wars, acts of war (whether war
is declared or not), terrorist acts, insurrections, riots, civil commotion, strikes, lockouts or other labour disturbances, acts of God or other acts, omissions or delays in acting by any administrative authority or other party. 

  
 28 

	 	14.2	The Parties shall notify each other in writing of any force majeure event which prevents such Party from performing its obligations under this Agreement. In the event a force majeure situation continues for more
than [***] months after notice is served, and is adversely effecting the performance of this Agreement, Ultragenyx will have the right, on [***] days advance written notice not to expire before the [***] month period to terminate this Agreement
without any further liability towards Rentschler. 

  

	15	Period for Limitation of Ultragenyx Claims 

 If not explicitly otherwise provided for in
this Agreement, but subject in all cases to Swiss law, claims by Ultragenyx in connection with this Agreement and/or the Services—regardless of their legal basis—are subject to a limitation period of [***] years after completion of the
Services. The foregoing period of limitation shall not apply to any claim brought by Ultragenyx to the extent resulting from or relating to (1) Rentschler’s breach of the warranty in Section 9.2.11, which instead shall be
subject to a limitation period of [***] years after completion of the Services, or (2) Rentschler’s gross negligence or willful misconduct, which shall [***]. 
  

	16	Applicable Law and Dispute Resolution 

  

	 	16.1	This Agreement shall be governed by and construed in accordance with the laws of Switzerland, without regards to its conflicts of law principles requiring the application of laws of another jurisdiction.

  

	 	16.2	Any dispute, controversy or claims arising out of or in connection with this Agreement, including disputes on its conclusion, binding effect, amendment and termination, shall be resolved exclusively by the
International Court of Arbitration of the International Chamber of Commerce and shall be finally settled under the Rules of Arbitration of the International Chamber of Commerce by three arbitrators appointed in accordance with the said Rules. The
seat of the arbitration shall be in Zurich, Switzerland. The arbitral proceedings shall be conducted in the English language. 

  

	17	Quality Agreement 

  

	 	17.1	Within [***] months of the Effective Date, the Parties will enter into a separate quality agreement (the “Quality Agreement”) with the Contract
No: 0467A7F4-15C7. 

  

	 	17.2	The Quality Agreement and its annexes supplement this Agreement. 

  

[***] Certain information in this document has been omitted and filed separately with the Securities and Exchange Commission. Confidential treatment has
been requested with respect to the omitted portions. 

  
 29 

	18	Miscellaneous 

 Amendment 

 

	 	18.1	No modification, extension or variation of this Agreement (or any document entered into pursuant to or in connection with this Agreement) shall be valid unless it is in writing and signed by or on behalf of each
of the Parties to this Agreement. For the avoidance of doubt, no modification or variation of this Agreement shall be valid if made by e-mail. 

  

	 	18.2	Unless expressly so agreed, no modification or variation of this Agreement shall constitute or be construed as a general waiver of any provisions of this Agreement, nor shall it affect any rights, obligations or
liabilities under this Agreement which have already accrued up to the date of such modification or waiver, and the rights and obligations of the Parties under this Agreement shall remain in full force and effect, except and only to the extent that
they are so modified or varied. 

 Assignment 
  

	 	18.3	Except as provided in Section 18.4, Ultragenyx shall not without the prior written consent of Rentschler (such consent not to be unreasonably withheld) assign this Agreement or any rights and
obligations under this Agreement. Each Party is entering into this Agreement as principal not agent, and may not enforce any of its rights under or in connection with this Agreement for the benefit of any other person. Any purported assignment in
breach of this Section shall confer no rights on the purported assignee. 

  

	 	18.4	Ultragenyx shall be entitled upon giving written notice to Rentschler to assign its rights under this Agreement to any member of Ultragenyx’s Group or to a third party which acquires all or substantially all
assets relating to the Product. Any assignment made pursuant to this Section 18.4 shall be subject to the following terms: 

  

	 	18.4.1	no such assignment shall relieve Ultragenyx of any of its accrued obligations under this Agreement; and 

  

	 	18.4.2	any such assignment shall be made on terms that the assignee acknowledges that Rentschler may continue to deal exclusively with Ultragenyx in respect of all matters relating to this Agreement at all times unless
and until the assignee notifies Rentschler in writing that it is exercising its rights as assignee. 

 Entire Agreement

  

	 	18.5	This Agreement, and the Schedules and documents referred to in it, constitutes the entire agreement and understanding of the Parties and supersedes any previous agreement between the Parties relating to the
subject matter of this Agreement. In the event of any conflict or ambiguity between this Agreement, the Quality Agreement, or any Schedule, this Agreement will control. 

  
 30 

 Waiver 
  

	 	18.6	In no event will any delay, failure or omission (in whole or in part) in enforcing, exercising or pursuing any right, power, privilege, claim or remedy conferred by or arising under this Agreement or by law, be
deemed to be or construed as a waiver of that or any other right, power, privilege, claim or remedy in respect of the circumstances in question, or operate so as to bar the enforcement of that, or any other right, power, privilege, claim or remedy,
in any other instance at any time or times subsequently. 

 Severability 

 

	 	18.7	If any provision of this Agreement shall be found by any court or administrative body of competent jurisdiction to be invalid or unenforceable, such invalidity or unenforceability shall not affect the other
provisions of this Agreement which shall remain in full force and effect. The Parties agree, in the circumstances referred to in this Section to attempt to substitute for any invalid or unenforceable provision a valid or enforceable provision which
achieves to the greatest extent possible the same effect as would have been achieved by the invalid or unenforceable provision. The obligations of the Parties under any invalid or unenforceable provision of this Agreement shall be suspended whilst
an attempt at such substitution is made. 

 Notices 

 

	 	18.8	Any notice given or made under this Agreement shall be in writing and signed by or on behalf of the Party giving it and shall be served by hand, delivering it or sending it by prepaid recorded or special delivery
post or prepaid international recorded airmail, to the address stated above or as notified in writing from time to time by the relevant Party to the other Party. 

Counterparts; Interpretation; Independent Contractors 
  

	 	18.9	This Agreement may be executed in any number of counterparts and by the Parties to it on separate counterparts, each of which shall be an original, but all of which together shall constitute one and the same
instrument. This Agreement is not effective until each Party has executed at least one counterpart. 

  

	 	18.10	This Agreement is in the English language only, which language shall be controlling in all respects, and all versions hereof in any other language shall be for accommodation only and shall not be binding on the Parties.
All terms used in this Agreement shall be interpreted in accordance with the corresponding Swiss legal terms. 

  
 31 

	 	18.11	The Parties shall perform their obligations under this Agreement as independent contractors, and nothing contained in this Agreement shall be construed to be inconsistent with such relationship or status. This Agreement
shall not constitute, create or in any way be interpreted as a joint venture or partnership of any kind. 

 Export
Control 
  

	 	18.12	This Agreement is made subject to any restrictions concerning the export of products or technical information from the United States of America or other countries which may be imposed upon or related to Ultragenyx or
Rentschler from time to time. Each Party agrees that it will not export, directly or indirectly, any technical information acquired from the other Party under this Agreement or any products using such technical information to a location or in a
manner that at the time of export requires an export license or other governmental approval, without first obtaining the written consent to do so from the appropriate agency or other governmental entity. 

THIS AGREEMENT has been executed by or on behalf of the Parties as of the date of the last signature shown below. 

Signed on behalf of 
 Rentschler Biotechnologie GmbH 

 

											
	Date: August 31, 2012	 		 	Date: August 31, 2012
					
	Signature:	 	/s/ Klaus Schoepe	 		 	Signature:	 	/s/ Thomas Sikloh
	Printed Name: Dr. Klaus Schoepe	 		 	Printed Name: Thomas Sikloh
	Position: SVP Client Relations	 		 	Position: SVP GMP-Operations
			
	 Signed on behalf of
 Ultragenyx
Pharmaceutical Inc.
	 		 	If second signature is required:
				
	Date: August 27, 2012	 		 	Date:	 	 
					
	Signature:	 	/s/ Thomas Kassberg	 		 	Signature: 	 	 
	Printed Name: Thomas Kassberg	 		 	Printed Name:	 	 
	Position: Chief Business Officer	 		 	Position:	 	 

  
 32EX-10.11

 Exhibit 10.11 

ULTRAGENYX PHARMACEUTICAL, INC. 

2011 EQUITY INCENTIVE PLAN 

ARTICLE 1 
 PURPOSE

 1.1 General. The purpose of the Ultragenyx Pharmaceutical, Inc. 2011 Equity Incentive Plan (the
“Plan”) is to promote the success and enhance the value of Ultragenyx Pharmaceutical, Inc., Inc., a Delaware corporation (the “Company”), by linking the personal interests of the members of the Board,
Employees and Consultants of the Company and any Parent or Subsidiary, to those of Company stockholders and by providing such individuals with an incentive for performance to generate returns to Company stockholders. The Plan is further intended to
provide flexibility to the Company in its ability to motivate, attract, and retain the services of members of the Board, Employees and Consultants of the Company and any Parent or Subsidiary upon whose judgment, interest, and special effort the
successful conduct of the Company’s operation is largely dependent. 
 ARTICLE 2 

DEFINITIONS AND CONSTRUCTION 

2.1 Definitions. The following words and phrases shall have the following meanings: 

(a) “Administrator” means the Board or a committee of the Board as described in Article 12. 

(b) “Award” means an Option, a Restricted Stock award, a Stock Appreciation Right award,
a Dividend Equivalents award, a Stock Payment award, or a Restricted Stock Unit award granted to a Participant pursuant to the Plan. 

(c) “Award Agreement” means any written or electronic agreement, contract, or other
instrument or document evidencing an Award. 
 (d) “Board” means the Board of Directors
of the Company. 
 (e) “Cause,” unless otherwise defined in an employment or services
agreement between the Participant and the Company or any Parent or Subsidiary, means (i) a Participant’s breach of any confidentiality or proprietary information agreement between the Participant and the Company or any Parent or
Subsidiary; (ii) a Participant’s conviction by, or entry of a plea of guilty or nolo contendere in, a court of competent and final jurisdiction for any crime involving moral turpitude or punishable by imprisonment in the jurisdiction
involved; (iii) a Participant’s commission of an act of fraud, whether prior to or subsequent to the date hereof upon the Company or any Parent or Subsidiary; (iv) a Participant’s continuing repeated willful failure or refusal to
perform his or her duties (including, without limitation, a Participant’s inability to perform his or her duties as a result of chronic alcoholism or drug addiction and/or as a result of any failure to comply with any laws, rules or regulations
of any governmental entity with respect to a Participant’s employment by the Company or any Parent or Subsidiary); (v) a Participant’s gross negligence, insubordination or material violation of any duty of loyalty to the Company or
any Parent or Subsidiary or any other material misconduct on the part of a  

 
Participant; (vi) a Participant’s intentional commission of any act which he or she knows (or reasonably should know) is likely to be materially detrimental to the
Company’s or any Parent’s or Subsidiary’s business or goodwill; or (vii) a Participant’s material breach of any other provision of any agreement between the Participant and the Company or any Parent or Subsidiary, provided
that termination of a Participant’s employment pursuant to this subsection (vii) shall not constitute valid termination for good cause unless such Participant shall have first received written notice from the Board or its designee stating
with specificity the nature of such breach and affording the Participant at least fifteen days to correct the breach alleged. 

The foregoing definition shall not in any way preclude or restrict the right of the Company or any successor or Parent or Subsidiary thereof
to discharge or dismiss any Participant in the service of such entity for any other acts or omissions, but such other acts or omissions shall not be deemed, for purposes of this Plan, to constitute grounds for termination for Cause. 

(f) “Change in Control” means and includes each of the following: 

(i) the acquisition, directly or indirectly, by any “person” or “group” (as those terms are defined in
Sections 3(a)(9), 13(d), and 14(d) of the Exchange Act and the rules thereunder) of “beneficial ownership” (as determined pursuant to Rule 13d-3 under the Exchange Act) of securities entitled to vote generally in the election of directors
(“voting securities”) of the Company that represent 50% or more of the combined voting power of the Company’s then outstanding voting securities, other than: 

(A) an acquisition by a trustee or other fiduciary holding securities under any employee benefit plan (or related trust)
sponsored or maintained by the Company or any person controlled by the Company or by any employee benefit plan (or related trust) sponsored or maintained by the Company or any person controlled by the Company, or 

(B) an acquisition of voting securities by the Company or a corporation owned, directly or indirectly, by the stockholders of
the Company in substantially the same proportions as their ownership of the stock of the Company, or 
 (C) an
acquisition of voting securities pursuant to a transaction described in subsection (iii) below that would not be a Change in Control under subsection (iii); 

Notwithstanding the foregoing, the following event shall not constitute an “acquisition” by any person or group for purposes
of this Section 2.1(e): an acquisition of the Company’s securities by the Company which causes the Company’s voting securities beneficially owned by a person or group to represent 50% or more of the combined voting power of the
Company’s then outstanding voting securities; provided, however, that if a person or group shall become the beneficial owner of 50% or more of the combined voting power of the Company’s then outstanding voting securities by reason
of share acquisitions by the Company as described above and shall, after such share acquisitions by the Company, become the beneficial owner of any additional voting securities of the Company, then such acquisition shall constitute a Change in
Control; or 

  
 2 

 (ii) during any period of two consecutive years, individuals who, at the
beginning of such period, constitute the Board together with any new director(s) (other than a director designated by a person who shall have entered into an agreement with the Company to effect a transaction described in subsections (a) or
(c) of this Section 2.1(e)) whose election by the Board or nomination for election by the Company’s stockholders was approved by a vote of at least two-thirds of the directors then still in office who either were directors at the
beginning of the two year period or whose election or nomination for election was previously so approved, cease for any reason to constitute a majority thereof; or 

(iii) the consummation by the Company (whether directly involving the Company or indirectly involving the Company through one
or more intermediaries) of a merger, consolidation, reorganization, or business combination, a sale or other disposition of all or substantially all of the Company’s assets, or the acquisition of assets or stock of another entity, in each case,
other than a transaction 
 (A) which results in the Company’s voting securities outstanding immediately
before the transaction continuing to represent (either by remaining outstanding or by being converted into voting securities of the Company or the person that, as a result of the transaction, controls, directly or indirectly, the Company or owns,
directly or indirectly, all or substantially all of the Company’s assets or otherwise succeeds to the business of the Company (the Company or such person, the “Successor Entity”)) directly or indirectly, at least 50% of
the combined voting power of the Successor Entity’s outstanding voting securities immediately after the transaction, and 

(B) after which no person or group beneficially owns voting securities representing 50% or more of the combined voting
power of the Successor Entity; provided, however, that no person or group shall be treated for purposes of this paragraph (iii) as beneficially owning 50% or more of combined voting power of the Successor Entity solely as a result of the
voting power held in the Company prior to the consummation of the transaction; or 
 (iv) the Company’s
stockholders approve a liquidation or dissolution of the Company. 
 For purposes of subsection (i) above, the calculation of voting
power shall be made as if the date of the acquisition were a record date for a vote of the Company’s stockholders, and for purposes of subsection (iii) above, the calculation of voting power shall be made as if the date of the consummation
of the transaction were a record date for a vote of the Company’s stockholders. 
 Notwithstanding the foregoing, a
transaction shall not constitute a “Change of Control” if: (i) its sole purpose is to change the state of the Company’s incorporation; (ii) its sole purpose is to create a holding company that will be owned in
substantially the same proportions by the persons who held the Company’s securities immediately before such transaction; (iii) it  

  
 3 

 
constitutes the Company’s initial public offering of its securities; or (iv) it is a transaction effected primarily for the purpose of financing the Company with cash (as
determined by the Administrator in its discretion and without regard to whether such transaction is effectuated by a merger, equity financing or otherwise). 

The Administrator shall have full and final authority, which shall be exercised in its sole discretion, to determine conclusively whether a
Change in Control of the Company has occurred pursuant to the above definition, and the date of the occurrence of such Change in Control and any incidental matters relating thereto. 

(g) “Code” means the Internal Revenue Code of 1986, as amended from time to time, and the regulations
issued thereunder. 
 (h) “Committee” means a committee of the Board described in Article 12. 

(i) “Consultant” means any consultant or adviser if: 

(i) The consultant or adviser renders bona fide services to the Company or any Parent or Subsidiary; and 

(ii) The services rendered by the consultant or adviser are not in connection with the offer or sale of securities in a
capital-raising transaction and do not directly or indirectly promote or maintain a market for the Company’s securities. 

(j) “Disability” means a permanent and total disability within the meaning of
Section 22(e)(3) of the Code, as it may be amended from time to time. 
 (k)
“Dividend Equivalents” means a right granted to a Participant pursuant to Article 8 to receive the equivalent value (in cash or Stock) of dividends paid on Stock. 

(l) “Eligible Individual” means any person who is a member of the Board, a Consultant or
an Employee, as determined by the Administrator. 
 (m) “Employee” means
any officer or other employee (as defined in accordance with Section 3401(c) of the Code) of the Company or any Parent or Subsidiary. 

(n) “Equity Restructuring” means a non-reciprocal transaction between the Company and its
stockholders, such as a stock dividend, stock split, spin-off, rights offering or recapitalization through a large, nonrecurring cash dividend, that affects the shares of Stock (or other securities of the Company) or the share price of Stock (or
other securities) and causes a change in the per share value of the Stock underlying outstanding Awards. 

(o) “Exchange Act” means the Securities Exchange Act of 1934, as amended from time to
time. 

  
 4 

 (p) “Fair Market Value” means, as of any
date, the value of Stock determined as follows: 
 (i) If the Stock is listed on any established stock exchange
or a national market system, including without limitation the Nasdaq National Market or The Nasdaq SmallCap Market of The Nasdaq Stock Market, its Fair Market Value shall be the closing sales price for such Stock as quoted on such exchange or system
for the last market trading day prior to the date of determination for which a closing sales price is reported, as reported in The Wall Street Journal or such other source as the Administrator deems reliable; 

(ii) If the Stock is regularly quoted by a recognized securities dealer but selling prices are not reported, its Fair Market
Value shall be the mean of the closing bid and asked prices for the Stock on the date prior to the date of determination as reported in The Wall Street Journal or such other source as the Administrator deems reliable; or 

(iii) In the absence of an established market for the Stock, the Fair Market Value thereof shall be determined in good faith by
the Administrator. 
 (q) “Good Reason” means a Participant’s voluntary
resignation following any one or more of the following that is effected without the Participant’s written consent: (i) a change in his or her position following the Change in Control that materially reduces his or her duties or
responsibilities, (ii) a reduction in his or her base salary following a Change in Control, unless the base salaries of all similarly situated individuals are similarly reduced, or (iii) a relocation of such Participant’s place of
employment of more than fifty miles following a Change in Control. However, if the term or concept of “Good Reason” has been defined in an agreement between a Participant and the Company or any successor or parent or Subsidiary thereof,
then “Good Reason” shall have the definition set forth in such agreement. 
 (r)
“Incentive Stock Option” means an Option that is intended to be an incentive stock option and meets the requirements of Section 422 of the Code or any successor provision thereto. 

(s) “Misconduct” means the commission of any commission of any act of fraud, embezzlement
or dishonesty by the Participant, any unauthorized use or disclosure by such person of confidential information or trade secrets of the Company (or any Parent or Subsidiary), or any other intentional misconduct by such person adversely affecting the
business or affairs of the Company (or any Parent or Subsidiary) in a material manner. The foregoing definition shall not in any way preclude or restrict the right of the Company (or any Parent or Subsidiary) to discharge or dismiss any Participant
or other person in the service of the Company (or any Parent or Subsidiary) for any other acts or omissions, but such other acts or omissions shall not be deemed, for purposes of the Plan, to constitute grounds for termination for Misconduct.

 (t) “Non-Employee Director” means a member of the Board who is not an
Employee. 
 (u) “Non-Qualified Stock Option” means an Option that is not
intended to be or otherwise does not qualify as an Incentive Stock Option. 

  
 5 

 (v) “Option” means a right granted to a
Participant pursuant to Article 5 of the Plan to purchase a specified number of shares of Stock at a specified price during specified time periods. An Option may be either an Incentive Stock Option or a Non-Qualified Stock Option. 

(w) “Parent” means any corporation in an unbroken chain of corporations ending with the
Company if each of the corporations other than the Company then owns stock possessing 50% or more of the total combined voting power of all classes of stock in one of the other corporations in such chain at the relevant time, including after the
Effective Date (as defined in Section 13.1). 
 (x) “Participant” means
any Eligible Individual who, as a member of the Board, an Employee or a Consultant, has been granted an Award pursuant to the Plan. 

(y) “Plan” means this Ultragenyx Pharmaceutical, Inc. 2011 Equity Incentive Plan, as it
may be amended from time to time. 
 (z) “Public Trading Date” means the
first date upon which the issuer is subject to the reporting requirements of Section 13 or 15(d)(2) of the Exchange Act. 

(aa) “Restricted Stock” means Stock awarded to a Participant pursuant to Article 6 that
is subject to certain restrictions and may be subject to risk of forfeiture or repurchase. 

(bb) “Restricted Stock Unit” means a right to receive a share of Stock during specified
time periods granted pursuant to Section 8.3. 
 (cc) “Securities Act”
means the Securities Act of 1933, as amended from time to time. 
 (dd) “Section 409A
Award” has the meaning set forth in Section 9.1. 
 (ee)
“Stock” means the common stock of the Company and such other securities of the Company that may be substituted for Stock pursuant to Article 11. 

(ff) “Stock Appreciation Right” or “SAR” means a right granted
pursuant to Article 7 to receive a payment equal to the excess of the Fair Market Value of a specified number of shares of Stock on the date the SAR is exercised over the Fair Market Value of such number of shares of Stock on the date the SAR was
granted as set forth in the applicable Award Agreement. 
 (gg) “Stock
Payment” means (a) a payment in the form of shares of Stock, or (b) an option or other right to purchase shares of Stock, as part of any bonus, deferred compensation or other arrangement, made in lieu of all or any portion of
the compensation, granted pursuant to Section 8.2. 
 (hh) “Subsidiary”
means any corporation or other entity of which a majority of the outstanding voting stock or voting power is beneficially owned directly or indirectly by the Company at the relevant time, including after the Effective Date (as defined in Section
13.1). 

  
 6 

 (ii) “Successor Entity” has the meaning set
forth in Section 2.1(f)(iii). 
 (jj) “Termination of Consultancy” means
the time when the engagement of a Participant as a Consultant to the Company or a Parent or Subsidiary is terminated for any reason, with or without cause, including, but not by way of limitation, by resignation, discharge, death or retirement, but
excluding terminations where there is a simultaneous commencement of employment with the Company or any Parent or Subsidiary. The Administrator, in its absolute discretion, shall determine the effect of all matters and questions relating to
Termination of Consultancy, including, but not by way of limitation, the question of whether a Termination of Consultancy resulted from a discharge for good cause, and all questions of whether a particular leave of absence constitutes a Termination
of Consultancy. Notwithstanding any other provision of the Plan, the Company or any Parent or Subsidiary has an absolute and unrestricted right to terminate a Consultant’s service at any time for any reason whatsoever, with or without cause,
except to the extent expressly provided otherwise in writing. 
 (kk) “Termination of
Directorship” shall mean the time when a Participant who is a Non-Employee Director ceases to be a member of the Board for any reason, including, but not by way of limitation, a termination by resignation, failure to be elected, death
or retirement. The Board, in its sole and absolute discretion, shall determine the effect of all matters and questions relating to Termination of Directorship with respect to Non- Employee Directors. 

(ll) “Termination of Employment” shall mean the time when the employee-employer
relationship between a Participant and the Company or any Parent or Subsidiary is terminated for any reason, with or without cause, including, but not by way of limitation, a termination by resignation, discharge, death, disability or retirement;
but excluding: (a) terminations where there is a simultaneous reemployment or continuing employment of a Participant by the Company or any Parent or Subsidiary, (b) at the discretion of the Administrator, terminations which result in a
temporary severance of the employee-employer relationship, and (c) terminations which are followed by the simultaneous establishment of a consulting relationship by the Company or a Parent or Subsidiary with the former employee. The
Administrator, in its absolute discretion, shall determine the effect of all matters and questions relating to Termination of Employment, including, but not by way of limitation, the question of whether a Termination of Employment resulted from a
discharge for good cause, and all questions of whether a particular leave of absence constitutes a Termination of Employment. 

(ii) “Termination of Service” shall mean the last to occur of a Participant’s
Termination of Employment, Termination of Directorship or Termination of Consultancy. A Participant shall not be deemed to have a Termination of Service merely because of a change in the capacity in which the Participant renders service to the
Company or any Parent or Subsidiary (i.e., a Participant who is an Employee becomes a Consultant) or a change in the entity for which the Participant renders such service (i.e., an Employee of the Company becomes an Employee of a Subsidiary), unless
following such change in capacity or service the Participant is no longer serving as an Employee, Non-Employee Director or Consultant of the Company or any Parent or Subsidiary. 

  
 7 

 ARTICLE 3 

SHARES SUBJECT TO THE PLAN 

3.1 Number of Shares. 

(a) Subject to Article 11, the aggregate number of shares of Stock which may be issued or transferred pursuant to Awards under
the Plan shall be 7,455,988 shares. 
 (b) To the extent that an Award terminates, expires, or lapses for any reason, any
shares of Stock subject to the Award shall again be available for the grant of an Award pursuant to the Plan. Additionally, any shares of Stock tendered or withheld to satisfy the grant or exercise price or tax withholding obligation pursuant to any
Award shall again be available for the grant of an Award pursuant to the Plan. If shares of Stock issued pursuant to Awards are forfeited by a Participant or repurchased by the Company pursuant to Section 6.3 hereof, such shares of Stock shall
become available for future grant under the Plan (unless the Plan has terminated). The payment of Dividend Equivalents in cash in conjunction with any outstanding Awards shall not be counted against the shares available for issuance under the Plan.

 (c) Notwithstanding the provisions of this Section 3.1, no shares of Stock may again be optioned, granted or awarded
if such action would cause an Incentive Stock Option to fail to qualify as an Incentive Stock Option under Section 422 of the Code. 

3.2 Stock Distributed. Any Stock distributed pursuant to an Award may consist, in whole or in part, of authorized and unissued Stock,
treasury Stock or, on and after the Public Trading Date, Stock purchased on the open market. 
 ARTICLE 4 

ELIGIBILITY AND PARTICIPATION 

4.1 Eligibility. Persons eligible to participate in this Plan include all Employees, Consultants and all members of the Board, as
determined by the Administrator. 
 4.2 Actual Participation. Subject to the provisions of the Plan, the Administrator may, from time
to time, select from among all Eligible Individuals those to whom Awards shall be granted and shall determine the nature and amount of each Award. No individual shall have any right to be granted an Award pursuant to this Plan. 

4.3 Foreign Participants. Notwithstanding any provision of the Plan to the contrary, in order to comply with the laws in other
countries in which the Company and its Parents or Subsidiaries operate or have Eligible Individuals, the Administrator, in its sole discretion, shall have the power and authority to: (i) determine which Parents or Subsidiaries shall be covered
by the Plan; (ii) determine which Eligible Individuals outside the United States are eligible to participate in the Plan; (iii) modify the terms and conditions of any Award granted to Eligible Individuals outside the United States to
comply with applicable foreign laws; (iv) establish subplans and modify exercise procedures and other terms and procedures, to the extent such actions may be necessary or advisable (any such subplans and/or modifications shall be attached to
this Plan as appendices); provided, however, that no such subplans and/or modifications shall increase the share limitation contained in Section 3.1 of the Plan; and (v) take any action, before

  
 8 

 
or after an Award is made, that it deems advisable to obtain approval or comply with any necessary local governmental regulatory exemptions or approvals. Notwithstanding the foregoing, the
Administrator may not take any actions hereunder, and no Awards shall be granted, that would violate the Exchange Act, the Code, any securities law or governing statute or any other applicable law. 

ARTICLE 5 
 STOCK OPTIONS

 5.1 General. The Administrator is authorized to grant Options to Eligible Individuals on the following terms and conditions:

 (a) Exercise Price. The exercise price per share of Stock subject to an Option shall be determined by the
Administrator and set forth in the Award Agreement; provided that the exercise price per share for any Option shall not be less than 100% of the Fair Market Value per share of the Stock on the date of the grant. 

(b) Time and Conditions of Exercise. The Administrator shall determine the time or times at which an Option may
be exercised in whole or in part; provided that the term of any Option granted under the Plan shall not exceed ten years. The Administrator shall also determine the performance or other conditions, if any, that must be satisfied before all or
part of an Option may be exercised. The Administrator may extend the term of any outstanding Option in connection with any Termination of Employment, Termination of Directorship or Termination of Consultancy of the Participant holding such Option,
or amend any other term or condition of such Option relating to such a Termination of Employment, Termination of Directorship or Termination of Consultancy. 

(c) Payment. The Administrator shall determine the methods, terms and conditions by which the exercise price of
an Option may be paid, and the form and manner of payment, including, without limitation, payment in the form of cash, a promissory note bearing interest at no less than such rate as shall then preclude the imputation of interest under the Code,
shares of Stock previously owned by the Participant or otherwise issuable upon exercise of the Option, or other property acceptable to the Administrator and payment through the delivery of a notice that the Participant has placed a market sell order
with a broker with respect to shares of Stock then issuable upon exercise of the Option, and that the broker has been directed to pay a sufficient portion of the net proceeds of the sale to the Company in satisfaction of the Option exercise price;
provided that payment of such proceeds is then made to the Company upon settlement of such sale, and the methods by which shares of Stock shall be delivered or deemed to be delivered to Participants. Notwithstanding any other provision of the
Plan to the contrary, no Participant who is a member of the Board or an “executive officer” of the Company within the meaning of Section 13(k) of the Exchange Act shall be permitted to pay the exercise price of an Option, or continue
any extension of credit with respect to the exercise price of an Option with a loan from the Company or a loan arranged by the Company, in any method which would violate Section 13(k) of the Exchange Act. 

  
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 (d) Evidence of Grant. All Options shall be evidenced by an Award
Agreement between the Company and the Participant. The Award Agreement shall include such additional provisions as may be specified by the Administrator. 

5.2 Incentive Stock Options. Incentive Stock Options may be granted only to employees (as defined in accordance with
Section 3401(c) of the Code) of the Company or a Subsidiary which constitutes a “subsidiary corporation” of the Company within Section 424(f) of the Code or a Parent which constitutes a “parent corporation” of the
Company within the meaning of Section 424(e) of the Code and the terms of any Incentive Stock Options granted pursuant to the Plan must comply with the following additional provisions of this Section 5.2 in addition to the requirements of
Section 5.1: 
 (a) Ten Percent Owners. An Incentive Stock Option shall be granted to any individual who,
at the date of grant, owns stock possessing more than ten percent of the total combined voting power of all classes of stock of the Company or any “subsidiary corporation” of the Company or “parent corporation” of the Company
(each within the meaning of Section 424 of the Code) only if such Option is granted at an exercise price per share that is not less than 110% of the Fair Market Value per share of the Stock on the date of the grant and the Option is exercisable
for no more than five years from the date of grant. 
 (b) Transfer Restriction. An Incentive Stock
Option shall not be transferable by the Participant other than by will or by the laws of descent or distribution. 

(c) Right to Exercise. During a Participant’s lifetime, an Incentive Stock Option may be exercised only by
the Participant. 
 (d) Failure to Meet Requirements. Any Option (or portion thereof) purported to be an
Incentive Stock Option which, for any reason, fails to meet the requirements of Section 422 of the Code shall be considered a Non- Qualified Stock Option. 

5.3 Early Exercisability. The Administrator may provide in the terms of a Participant’s Award Agreement that the Participant may,
at any time before the Participant’s status as an Employee, member of the Board or Consultant terminates, exercise the Option(s) granted to such Participant in whole or in part prior to the full vesting of the Option(s); provided, however,
shares of Stock acquired upon exercise of an Option which has not fully vested may be subject to any forfeiture, transfer or other restrictions as the Administrator may determine in its sole discretion. 

5.4 Paperless Exercise. In the event that the Company establishes, for itself or using the services of a third party, an automated
system for the exercise of Options, such as a system using an internet website or interactive voice response, then the paperless exercise of Options by a Participant may be permitted through the use of such an automated system. 

ARTICLE 6 
 RESTRICTED
STOCK AWARDS 
 6.1 Grant of Restricted Stock. The Administrator is authorized to make Awards of Restricted Stock to any Eligible
Individual selected by the Administrator in such amounts and subject to such terms and conditions as determined by the Administrator. All Awards of Restricted Stock shall be evidenced by an Award Agreement. 

  
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 6.2 Issuance and Restrictions. Restricted Stock shall be subject to such repurchase
restrictions, forfeiture restrictions, restrictions on transferability and other restrictions as the Administrator may impose (including, without limitation, limitations on the right to vote Restricted Stock or the right to receive dividends on the
Restricted Stock). These restrictions may lapse separately or in combination at such times, pursuant to such circumstances or in such installments or otherwise as the Administrator determines at the time of the grant of the Award or thereafter. 

6.3 Repurchase or Forfeiture. Except as otherwise determined by the Administrator at the time of the grant of the Award or thereafter,
upon a Participant’s Termination of Service during the applicable restriction period, Restricted Stock that is at that time subject to restrictions shall be forfeited or subject to repurchase by the Company (or its assignee) under such terms as
the Administrator shall determine; provided, however, that the Administrator may (a) provide in any Restricted Stock Award Agreement that restrictions or forfeiture conditions relating to Restricted Stock will be waived in whole or in
part in the event of a Participant’s Termination of Service, and (b) in other cases waive in whole or in part restrictions or forfeiture conditions relating to Restricted Stock. 

6.4 Certificates for Restricted Stock. Restricted Stock granted pursuant to the Plan may be evidenced in such manner as the
Administrator shall determine. If certificates representing shares of Restricted Stock are registered in the name of the Participant, certificates must bear an appropriate legend referring to the terms, conditions, and restrictions applicable to
such Restricted Stock, and the Company may, at its discretion, retain physical possession of the certificate until such time as all applicable restrictions lapse or the Award Agreement may provide that the shares shall be held in escrow by an escrow
agent designated by the Company. 
 ARTICLE 7 

STOCK APPRECIATION RIGHTS 

7.1 Grant of Stock Appreciation Rights. A Stock Appreciation Right may be granted to any Eligible Individual selected by the
Administrator. A Stock Appreciation Right shall be subject to such terms and conditions not inconsistent with the Plan as the Administrator shall impose and shall be evidenced by an Award Agreement. 

7.2 Terms of Stock Appreciation Rights. 

(a) A Stock Appreciation Right shall have a term set by the Administrator. A Stock Appreciation Right shall be exercisable in
such installments as the Administrator may determine. A Stock Appreciation Right shall cover such number of shares of Stock as the Administrator may determine. The exercise price per share of Stock subject to each Stock Appreciation Right shall be
set by the Administrator. 
 (b) A Stock Appreciation Right shall entitle the Participant (or other person entitled to
exercise the Stock Appreciation Right pursuant to the Plan) to exercise all or a specified portion of the Stock Appreciation Right (to the extent then exercisable pursuant to its 

  
 11 

 
terms) and to receive from the Company an amount determined by multiplying (i) the amount (if any) by which the Fair Market Value of a share of Stock on the date of exercise of the Stock
Appreciation Right exceeds the exercise price per share of the Stock Appreciation Right, by (ii) the number of shares of Stock with respect to which the Stock Appreciation Right shall have been exercised, subject to any limitations the
Administrator may impose. 
 7.3 Payment and Limitations on Exercise. 

(a) Subject to Sections 7.3(b) and (c), payment of the amounts determined under Section 7.2(b) above shall be in cash, in
Stock (based on its Fair Market Value as of the date the Stock Appreciation Right is exercised) or a combination of both, as determined by the Administrator. 

(b) To the extent payment for a Stock Appreciation Right is to be made in cash, the Award Agreement shall, to the extent
necessary to comply with the requirements of Section 409A of the Code, specify the date of payment, which may be different than the date of exercise of the Stock Appreciation Right. If the date of payment for a Stock Appreciation Right is later
than the date of exercise, the Award Agreement may specify that the Participant be entitled to earnings on such amount until paid. 

(c) To the extent any payment under Section 7.2(b) is effected in Stock, it shall be made subject to satisfaction of all
provisions of Article 5 above pertaining to Options. 
 ARTICLE 8 

OTHER TYPES OF AWARDS 
 8.1
Dividend Equivalents. Any Eligible Individual selected by the Administrator may be granted Dividend Equivalents based on the dividends declared on the shares of Stock that are subject to any Award, to be credited as of dividend payment dates,
during the period between the date the Award is granted and the date the Award is exercised, vests or expires, as determined by the Administrator. Such Dividend Equivalents shall be converted to cash or additional shares of Stock by such formula and
at such time and subject to such limitations as may be determined by the Administrator. 
 8.2 Stock Payments. Any Eligible
Individual selected by the Administrator may receive Stock Payments in the manner determined from time to time by the Administrator; provided that, unless otherwise determined by the Administrator, such Stock Payments shall be made in lieu of
base salary, bonus or other cash compensation otherwise payable to such Eligible Individual. The number of shares shall be determined by the Administrator and may be based upon the Performance Goals or other specific performance goals determined
appropriate by the Administrator. 
 8.3 Restricted Stock Units. The Administrator is authorized to make Awards of Restricted Stock
Units to any Eligible Individual selected by the Administrator in such amounts and subject to such terms and conditions as determined by the Administrator. At the time of grant, the Administrator shall specify the date or dates on which the
Restricted Stock Units shall become fully vested and nonforfeitable, and may specify such conditions to vesting as it deems appropriate. Alternatively, Restricted Stock Units may become fully vested and nonforfeitable

  
 12 

 
pursuant to the satisfaction of one or more Performance Goals or other specific performance goals as the Administrator determines to be appropriate at the time of the grant of the Restricted
Stock Units or thereafter, in each case on a specified date or dates or over any period or periods determined by the Administrator. At the time of grant, the Administrator shall specify the maturity date applicable to each grant of Restricted Stock
Units which shall be no earlier than the vesting date or dates of the Award and, to the extent permitted by the Administrator, may be determined at the election of the Eligible Individual to whom the Award is granted. On the maturity date, the
Company shall transfer to the Participant one unrestricted, fully transferable share of Stock for each Restricted Stock Unit that is vested and scheduled to be distributed on such date and not previously forfeited. The Administrator shall specify
the purchase price, if any, to be paid by the Participant to the Company for such shares of Stock. 
 8.4 Term. Except as otherwise
provided herein, the term of any Award of Performance Shares, Dividend Equivalents, Stock Payments or Restricted Stock Units shall be set by the Administrator in its discretion. 

8.5 Exercise or Purchase Price. The Administrator may establish the exercise or purchase price, if any, of any Award of Restricted
Stock Units or Stock Payments; provided, however, that such price shall not be less than the par value of a share of Stock on the date of grant, unless otherwise permitted by applicable state law. 

8.6 Form of Payment. Payments with respect to any Awards granted under Sections 8.1, 8.2 or 8.3 shall be made in cash, in Stock or a
combination of both, as determined by the Administrator. 
 8.7 Award Agreement. All Awards under this Article 8 shall be subject to
such additional terms and conditions as determined by the Administrator and shall be evidenced by a written Award Agreement. 
 ARTICLE 9

 COMPLIANCE WITH SECTION 409A OF THE CODE 

9.1 Awards subject to Code Section 409A. Any Award that constitutes, or provides for, a deferral of compensation subject to
Section 409A of the Code (a “Section 409A Award”) shall satisfy the requirements of Section 409A of the Code and this Article 9, to the extent applicable. The Award Agreement with respect to a Section 409A
Award shall incorporate the terms and conditions required by Section 409A of the Code and this Article 9. 
 9.2 Distributions under
a Section 409A Award. 
 (a) Subject to subsection (b), any shares of Stock or other property or amounts to be paid
or distributed upon the grant, issuance, vesting, exercise or payment of a Section 409A Award shall be distributed in accordance with the requirements of Section 409A(a)(2) of the Code, and shall not be distributed earlier than: 

(i) the Participant’s separation from service, as determined by the Secretary of the Treasury; 

  
 13 

 (ii) the date the Participant becomes disabled; 

(iii) the Participant’s death; 

(iv) a specified time (or pursuant to a fixed schedule) specified under the Award Agreement at the date of the deferral
compensation; 
 (v) to the extent provided by the Secretary of the Treasury, a change in the ownership or effective control
of the Company or a Parent or Subsidiary, or in the ownership of a substantial portion of the assets of the Company or a Parent or Subsidiary; or 

(vi) the occurrence of an unforeseeable emergency with respect to the Participant. 

(b) In the case of a Participant who is a “specified employee,” the requirement of paragraph (a)(i) shall be met only
if the distributions with respect to the Section 409A Award may not be made before the date which is six months after the Participant’s separation from service (or, if earlier, the date of the Participant’s death). For purposes of
this subsection (b), a Participant shall be a “specified employee” if such Participant is a key employee (as defined in Section 416(i) of the Code without regard to paragraph (5) thereof) of a corporation any stock of which is
publicly traded on an established securities market or otherwise, as determined under Section 409A(a)(2)(B)(i) of the Code and the Treasury Regulations thereunder. 

(c) The requirement of paragraph (a)(vi) shall be met only if, as determined under Treasury Regulations under
Section 409A(a)(2)(B)(ii) of the Code, the amounts distributed with respect to the unforeseeable emergency do not exceed the amounts necessary to satisfy such unforeseeable emergency plus amounts necessary to pay taxes reasonably anticipated as
a result of the distribution, after taking into account the extent to which such unforeseeable emergency is or may be relieved through reimbursement or compensation by insurance or otherwise or by liquidation of the Participant’s assets (to the
extent the liquidation of such assets would not itself cause severe financial hardship). 
 (d) For purposes of this Section,
the terms specified therein shall have the respective meanings ascribed thereto under Section 409A of the Code and the Treasury Regulations thereunder. 

9.3 Prohibition on Acceleration of Benefits. The time or schedule of any distribution or payment of any shares of Stock or other
property or amounts under a Section 409A Award shall not be accelerated, except as otherwise permitted under Section 409A(a)(3) of the Code and the Treasury Regulations thereunder. 

9.4 Elections under Section 409A Awards. 

(a) Any deferral election provided under or with respect to an Award to any Eligible Individual, or to the Participant holding
a Section 409A Award, shall satisfy the requirements of Section 409A(a)(4)(B) of the Code, to the extent applicable, and, except as otherwise permitted under paragraph (i) or (ii) below, any such deferral election with respect to
compensation for services performed during a taxable year shall be made not later than the close of the preceding taxable year, or at such other time as provided in Treasury Regulations. 

(i) In the case of the first year in which an Eligible Individual or a Participant holding a Section 409A Award, becomes
eligible to participate in the Plan, any such deferral election may be made with respect to services to be performed subsequent to the election with thirty days after the date the Eligible Individual, or the Participant holding a Section 409A
Award, becomes eligible to participate in the Plan, as provided under Section 409A(a)(4)(B)(ii) of the Code. 

  
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 (ii) In the case of any performance-based compensation based on services
performed by an Eligible Individual, or the Participant holding a Section 409A Award, over a period of at least twelve months, any such deferral election may be made no later than six months before the end of the period, as provided under
Section 409A(a)(4)(B)(iii) of the Code. 
 (b) In the event that a Section 409A Award permits, under a subsequent
election by the Participant holding such Section 409A Award, a delay in a distribution or payment of any shares of Stock or other property or amounts under such Section 409A Award, or a change in the form of distribution or payment, such
subsequent election shall satisfy the requirements of Section 409A(a)(4)(C) of the Code, and: 
 (i) such subsequent
election may not take effect until at least twelve months after the date on which the election is made, 
 (ii) in the case
such subsequent election relates to a distribution or payment not described in Section 9.2(a)(ii), (iii) or (vi), the first payment with respect to such election may be deferred for a period of not less than five years from the date such
distribution or payment otherwise would have been made, and 
 (iii) in the case such subsequent election relates to a
distribution or payment described in Section 9.2(a)(iv), such election may not be made less than twelve months prior to the date of the first scheduled distribution or payment under Section 9.2(a)(iv). 

9.5 Compliance in Form and Operation. A Section 409A Award, and any election under or with respect to such Section 409A
Award, shall comply in form and operation with the requirements of Section 409A of the Code and the Treasury Regulations thereunder. 

ARTICLE 10 
 PROVISIONS
APPLICABLE TO AWARDS 
 10.1 Stand-Alone and Tandem Awards. Awards granted pursuant to the Plan may, in the discretion of the
Administrator, be granted either alone, in addition to, or in tandem with, any other Award granted pursuant to the Plan. Awards granted in addition to or in tandem with other Awards may be granted either at the same time as or at a different time
from the grant of such other Awards. 

  
 15 

 10.2 Award Agreement. Awards under the Plan shall be evidenced by Award Agreements that
set forth the terms, conditions and limitations for each Award which may include the term of an Award, the provisions applicable in the event of the Participant’s Termination of Service, and the Company’s authority to unilaterally or
bilaterally amend, modify, suspend, cancel or rescind an Award. 
 10.3 Limits on Transfer. 

(a) Except as otherwise provided by the Administrator pursuant to Section 10.3(b), no right or interest of a Participant
in any Award may be pledged, encumbered, or hypothecated to or in favor of any party other than the Company or a Parent or Subsidiary, or shall be subject to any lien, obligation, or liability of such Participant to any other party other than the
Company or a Parent or Subsidiary. Except as otherwise provided by the Administrator pursuant to Section 10.3(b), no Award shall be assigned, transferred, or otherwise disposed of by a Participant other than by will or the laws of descent and
distribution, unless and until such Award has been exercised, or the shares underlying such Award have been issued, and all restrictions applicable to such shares have lapsed. 

(b) Notwithstanding Section 10.3(a), the Administrator, in its sole discretion, may permit an Award (other
than an Incentive Stock Option) to be transferred to, exercised by and paid to any one or more Permitted Transferees (as defined below), subject to the following terms and conditions: (i) an Award transferred to a Permitted Transferee shall not
be assignable or transferable by the Permitted Transferee other than by will or the laws of descent and distribution; (ii) any Award which is transferred to a Permitted Transferee shall continue to be subject to all the terms and conditions of
the Award as applicable to the original Participant (other than the ability to further transfer the Award); and (iii) the Participant and the Permitted Transferee shall execute any and all documents requested by the Administrator, including,
without limitation documents to (A) confirm the status of the transferee as a Permitted Transferee, (B) satisfy any requirements for an exemption for the transfer under applicable federal and state securities laws and (C) evidence the
transfer. For purposes of this Section 10.3(b), “Permitted Transferee” shall mean, with respect to a Participant, any child, stepchild, grandchild, parent, stepparent, grandparent, spouse, former spouse, sibling, niece,
nephew, mother-in-law, father-in-law, son-in-law, daughter-in-law, brother-in-law, or sister-in-law, including adoptive relationships, any person sharing the Participant’s household (other than a tenant or employee), a trust in which these
persons (or the Participant) control the management of assets, and any other entity in which these persons (or the Participant) own more than fifty percent of the voting interests, or any other transferee specifically approved by the
Administrator. 
 10.4 Beneficiaries. Notwithstanding Section 10.3, a Participant may, in the manner determined by
the Administrator, designate a beneficiary to exercise the rights of the Participant and to receive any distribution with respect to any Award upon the Participant’s death. A beneficiary, legal guardian, legal representative, or other person
claiming any rights pursuant to the Plan is subject to all terms and conditions of the Plan and any Award Agreement applicable to the Participant, except to the extent the Plan and Award Agreement otherwise provide, and to any additional
restrictions deemed necessary or appropriate by the Administrator. If the Participant is married and resides in a community property state, a designation of a person other 

  
 16 

 
than the Participant’s spouse as his or her beneficiary with respect to more than 50% of the Participant’s interest in the Award shall not be effective without the prior written consent
of the Participant’s spouse. If no beneficiary has been designated or survives the Participant, payment shall be made to the person entitled thereto pursuant to the Participant’s will or the laws of descent and distribution. Subject to the
foregoing, a beneficiary designation may be changed or revoked by a Participant at any time provided the change or revocation is filed with the Administrator. 

10.5 Stock Certificates; Book Entry Procedures. 

(a) Notwithstanding anything herein to the contrary, the Company shall not be required to issue or deliver any certificates
evidencing shares of Stock pursuant to the exercise or purchase of any Award, unless and until the Board has determined, with advice of counsel, that the issuance and delivery of such certificates is in compliance with all applicable laws,
regulations of governmental authorities and, if applicable, the requirements of any exchange on which the shares of Stock are listed or traded. All Stock certificates delivered pursuant to the Plan are subject to any stop-transfer orders and other
restrictions as the Administrator deems necessary or advisable to comply with federal, state, or foreign jurisdiction, securities or other laws, rules and regulations and the rules of any national securities exchange or automated quotation system on
which the Stock is listed, quoted, or traded. The Administrator may place legends on any Stock certificate to reference restrictions applicable to the Stock. In addition to the terms and conditions provided herein, the Administrator may require that
a Participant make such reasonable covenants, agreements, and representations as the Administrator, in its discretion, deems advisable in order to comply with any such laws, regulations, or requirements. The Administrator shall have the right to
require any Participant to comply with any timing or other restrictions with respect to the settlement or exercise of any Award, including a window-period limitation, as may be imposed in the discretion of the Administrator. 

(b) Notwithstanding any other provision of the Plan, unless otherwise determined by the Administrator or required by applicable
law, rule or regulation, the Company shall not deliver to any Participant certificates evidencing shares of Stock issued in connection with any Award or exercise of any Award and instead such shares of Stock will be recorded in the books of the
Company (or as applicable, its transfer agent or stock plan administrator). 
 ARTICLE 11 

CHANGES IN CAPITAL STRUCTURE 

11.1 Adjustments. 

(a) In the event of any combination or exchange of shares, merger, consolidation, distribution of Company assets to
stockholders (other than normal cash dividends), or any other corporate event affecting the Stock or the share price of the Stock, other than an Equity Restructuring, the Administrator shall make such proportionate adjustments to reflect such change
with respect to (i) the aggregate number and type of shares that may be issued under the Plan (including, but not limited to, adjustments of the limitation in Section 3.1); (ii) the terms and conditions of any outstanding Awards
(including, without limitation, any applicable performance targets or criteria with respect thereto); and (iii) the grant or exercise price per share for any outstanding Awards under the Plan. 

  
 17 

 (b) In the event of any transaction or event described in Section 11.1(a) or
any unusual or nonrecurring transactions or events affecting the Company, any affiliate of the Company, or the financial statements of the Company or any affiliate (including without limitation any Change in Control), other than an Equity
Restructuring, or of changes in applicable laws, regulations or accounting principles, and whenever the Administrator determines that such action is appropriate in order to prevent the dilution or enlargement of the benefits or potential benefits
intended to be made available under the Plan or with respect to any Award under the Plan, to facilitate such transactions or events or to give effect to such changes in laws, regulations or principles, the Administrator, in its sole discretion and
on such terms and conditions as it deems appropriate, either by amendment of the terms of any outstanding Awards or by action taken prior to the occurrence of such transaction or event and either automatically or upon the Participant’s request,
is hereby authorized to take any one or more of the following actions: 
 (i) To provide for either (A) termination of
any such Award in exchange for an amount of cash and/or other property, if any, equal to the amount that would have been received upon the exercise of such Award or realization of the Participant’s rights (and, for the avoidance of doubt, if as
of the date of the occurrence of the transaction or event described in this Section 11.1(b) the Administrator determines in good faith that no amount would have been attained upon the exercise of such Award or realization of the
Participant’s rights, then such Award may be terminated by the Company without payment) or (B) the replacement of such Award with other rights or property selected by the Administrator in its sole discretion; 

(ii) To provide that such Award be assumed by the successor or survivor entity, or a parent or subsidiary thereof, or shall be
substituted for by similar options, rights or awards covering the stock of the successor or survivor entity, or a parent or subsidiary thereof, with appropriate adjustments as to the number and kind of shares and prices; 

(iii) To make adjustments in the number and type of shares of Stock (or other securities or property) subject to outstanding
Awards, and in the number and kind of outstanding Restricted Stock or Restricted Stock Units and/or in the terms and conditions of (including the grant or exercise price), and the criteria included in, outstanding options, rights and awards, and
options, rights and awards which may be granted in the future; 
 (iv) To provide that such Award shall be exercisable or
payable or fully vested with respect to all shares covered thereby, notwithstanding anything to the contrary in the Plan or the applicable Award Agreement; and 

(v) To provide that the Award cannot vest, be exercised or become payable after such event. 

  
 18 

 (c) In connection with the occurrence of any Equity Restructuring, and
notwithstanding anything to the contrary in Sections 11.1(a) and 11.1(b): 
 (i) The number and type of securities subject to
each outstanding Award and the exercise price or grant price thereof, if applicable, will be proportionately adjusted by the Administrator as the Administrator deems appropriate to reflect such Equity Restructuring. The adjustments provided under
this Section 11(c)(i) shall be nondiscretionary and shall be final and binding on the affected Participant and the Company. 

(ii) The Administrator shall make such proportionate adjustments, if any, as the Administrator in its discretion may deem
appropriate to reflect such Equity Restructuring with respect to the aggregate number and kind of shares that may be issued under the Plan (including, but not limited to, adjustments of the limitations in Article 3). 

11.2 Acceleration Upon a Change in Control. 

(a) Notwithstanding anything to the contrary contained in Section 11.1, and except as may otherwise be provided in any
applicable Award Agreement or other written agreement entered into between the Company and a Participant, if a Change in Control occurs and a Participant’s Awards are not continued, converted, assumed or replaced by (i) the Company or a
Parent or Subsidiary, or (ii) a Successor Entity, such Awards shall become fully exercisable and/or payable, as applicable, and all forfeiture, repurchase and other restrictions on such Awards shall lapse immediately prior to such Change in
Control. Upon, or in anticipation of, a Change in Control, the Administrator may cause any and all Awards outstanding hereunder to terminate at a specific time in the future, including without limitation, the date of such Change in Control, and
shall give each Participant the right to exercise such Awards during a period of time as the Administrator, in its sole and absolute discretion, shall determine. The Administrator shall have sole discretion to determine whether an Award has been
continued, converted, assumed or replaced in connection with a Change in Control. 
 (b) Except as otherwise provided in the
Agreement evidencing the Award, any such Awards that are continued, converted, assumed, or replaced by (i) the Company or a Parent or Subsidiary of the Company, or (ii) a Successor Entity, in a Change in Control and do not otherwise
accelerate at that time shall become fully exercisable and/or payable, as applicable, and all forfeiture, repurchase and other restrictions on such Awards shall lapse in the event that the Participant has a Termination of Employment, Termination of
Directorship or Termination of Consultancy (i) in connection with the Change in Control or (ii) subsequently within twelve months following such Change in Control, unless such termination is by reason of the Participant’s discharge by
the Company or a Parent or Subsidiary or a Successor Entity for Cause or by reason of the Participant’s voluntary resignation without Good Reason. 

11.3 No Other Rights. Except as expressly provided in the Plan or pursuant to action of the Administrator under the Plan, no
Participant shall have any rights by reason of any subdivision or consolidation of shares of stock of any class, the payment of any dividend, any increase or decrease in the number of shares of stock of any class or any dissolution, liquidation,
merger, or consolidation of the Company or any other corporation. Except as expressly provided in the Plan or pursuant to action of the Administrator under the Plan, no issuance by the Company of shares of stock of any class, or securities
convertible into shares of stock of any class, shall affect, and no adjustment by reason thereof shall be made with respect to, the number of shares of Stock subject to an Award or the grant or exercise price of any Award. 

  
 19 

 ARTICLE 12 

ADMINISTRATION 
 12.1
Administrator. The Plan shall be administered by the Board. The Board may delegate administration of the Plan to a Committee or Committees of one or more members of the Board, and the term “Administrator” shall apply
to any person or persons who at the time have the authority to administer the Plan. If administration is delegated to a Committee, the Committee shall have, in connection with the administration of the Plan, the powers theretofore possessed by the
Board, including the power to delegate to a subcommittee any of the administrative powers the Committee is authorized to exercise (and references in this Plan to the Board shall thereafter be to the Committee or subcommittee), subject, however, to
such resolutions, not inconsistent with the provisions of the Plan, as may be adopted from time to time by the Board. Notwithstanding the foregoing, however, from and after the Public Trading Date, a Committee of the Board shall administer the Plan
and such Committee shall consist solely of two or more members of the Board each of whom is an “outside director,” within the meaning of Section 162(m) of the Code and a Non-Employee Director. Notwithstanding the foregoing:
(a) the full Board, acting by a majority of its members in office, shall conduct the general administration of the Plan with respect to all Awards granted to Non-Employee Directors and for purposes of such Awards the term
“Administrator” as used in this Plan shall be deemed to refer to the Board, and (b) the Board or the Committee may delegate its authority hereunder to the extent permitted by Section 12.5. In its sole discretion,
the Board may at any time and from time to time exercise any and all rights and duties of the Administrator under the Plan except with respect to matters which, following the Public Trading Date, are required to be determined in the sole discretion
of the Committee under Rule 16b-3 under the Exchange Act or Section 162(m) of the Code, or any regulations or rules issued thereunder. Committee members may resign at any time by delivering written notice to the Board. Vacancies in the
Committee may only be filled by the Board. 
 12.2 Action by the Administrator. A majority of the members of the Administrator shall
constitute a quorum. The acts of a majority of the members of the Administrator present at any meeting at which a quorum is present, and, subject to applicable law, acts approved in writing by a majority of the members of the Administrator in lieu
of a meeting, shall be deemed the acts of the Administrator. Each member of the Administrator is entitled to, in good faith, rely or act upon any report or other information furnished to that member by any officer or other employee of the Company or
any Parent or Subsidiary, the Company’s independent certified public accountants, or any executive compensation consultant or other professional retained by the Company to assist in the administration of the Plan. 

12.3 Authority of Administrator. Subject to any specific designation in the Plan, the Administrator has the exclusive power, authority
and discretion to: 
 (a) Designate Eligible Individuals to receive Awards; 

(b) Determine the type or types of Awards to be granted to each Eligible Individual; 

  
 20 

 (c) Determine the number of Awards to be granted and the number of shares of
Stock to which an Award will relate; 
 (d) Determine the terms and conditions of any Award granted pursuant to the Plan,
including, but not limited to, the exercise price, grant price, or purchase price, any reload provision, any restrictions or limitations on the Award, any schedule for lapse of forfeiture restrictions or restrictions on the exercisability of an
Award, and accelerations or waivers thereof, any provisions related to non-competition and recapture of gain on an Award, based in each case on such considerations as the Administrator in its sole discretion determines; 

(e) Determine whether, to what extent, and pursuant to what circumstances an Award may be settled in, or the exercise price of
an Award may be paid in, cash, Stock, other Awards, or other property, or an Award may be canceled, forfeited, or surrendered; 

(f) Prescribe the form of each Award Agreement, which need not be identical for each Participant; 

(g) Decide all other matters that must be determined in connection with an Award; 

(h) Establish, adopt, or revise any rules and regulations as it may deem necessary or advisable to administer the Plan; 

(i) Interpret the terms of, and any matter arising pursuant to, the Plan or any Award Agreement; and 

(j) Make all other decisions and determinations that may be required pursuant to the Plan or as the Administrator deems
necessary or advisable to administer the Plan. 
 11.4 Decisions Binding. The Administrator’s interpretation of the Plan, any
Awards granted pursuant to the Plan, any Award Agreement and all decisions and determinations by the Administrator with respect to the Plan are final, binding, and conclusive on all parties. 

11.5 Delegation of Authority. Within the scope of such authority, the Board or the Committee may delegate to a committee of one or more
members of the Board or one or more officers of the Company the authority to grant or amend Awards to Participants other than Eligible Individuals who are either (a) “covered employees” at the time of recognition of income resulting
from such Awards, and/or (b) persons with respect to whom the Company wishes to comply with Section 162(m) of the Code and/or (c) subject to Section 16 of the Exchange Act and/or (d) officers of the Company or members of the
Board to whom authority to grant or amend Awards has been delegated pursuant to this Section 12.5. At all times, the delegate(s) appointed under this Section 12.5 shall serve in such capacity at the pleasure of the Board or the Committee.

  
 21 

 ARTICLE 13 

EFFECTIVE AND EXPIRATION DATE 

13.1 Effective Date. The Plan will be effective on the date of the Board’s initial adoption of the Plan (the “Effective
Date”). The Plan will be submitted for the approval of the Company’s stockholders within twelve months after the Effective Date. Awards may be granted or awarded prior to such stockholder approval, provided that such Awards shall
not be exercisable, shall not vest and the restrictions thereon shall not lapse prior to the time when the Plan is approved by the stockholders, and provided further that if such approval has not been obtained at the end of said twelve-month period,
all Awards previously granted or awarded under the Plan shall thereupon be canceled and become null and void. 
 13.2 Expiration
Date. The Plan will expire on, and no Award may be granted pursuant to the Plan after, the tenth anniversary of the earlier of (i) the Effective Date or (ii) the date this Plan is approved by the Company’s stockholders. Any Awards
that are outstanding on the tenth anniversary of the Effective Date shall remain in force according to the terms of the Plan and the applicable Award Agreement. 

ARTICLE 14 
 AMENDMENT,
MODIFICATION, AND TERMINATION 
 14.1 Amendment, Modification, and Termination. The Board may terminate, amend or modify the Plan
at any time and from time to time; provided, however, that to the extent necessary to comply with any applicable law, regulation, or stock exchange rule, the Company shall obtain stockholder approval of any Plan amendment in such a manner and
to such a degree as required. The Administrator shall have the authority to effect, at any time and from time to time, with the consent of the affected Option holders, the cancellation of any or all outstanding Awards under the Plan and to grant in
substitution therefor new Awards covering the same or different number of shares of Stock and with a different or no exercise price per share. 

14.2 Awards Previously Granted. No termination, amendment, or modification of the Plan shall adversely affect in any material way any
Award previously granted pursuant to the Plan without the prior written consent of the Participant. 
 ARTICLE 15 

GENERAL PROVISIONS 
 15.1
No Rights to Awards. No Participant, Employee, or other person shall have any claim to be granted any Award pursuant to the Plan, and neither the Company nor the Administrator is obligated to treat Participants, Employees, and other persons
uniformly. 
 15.2 No Stockholder Rights. Except as otherwise provided herein, a Participant shall have none of the rights of a
stockholder with respect to shares of Stock covered by any Award until the Participant becomes the record owner of such shares of Stock. 

15.3 Withholding. The Company or any Parent or Subsidiary shall have the authority and the right to deduct or withhold, or require a
Participant to remit to the Company an amount sufficient to satisfy federal, state, local and foreign taxes (including the Participant’s employment 

  
 22 

 
tax obligations) required by law to be withheld with respect to any taxable event concerning a Participant arising as a result of this Plan. The Administrator may in its discretion and in
satisfaction of the foregoing requirement allow a Participant to elect to have the Company or a Parent or Subsidiary, as applicable, withhold shares of Stock otherwise issuable under an Award (or allow the return of shares of Stock) having a Fair
Market Value equal to the sums required to be withheld. Notwithstanding any other provision of the Plan, the number of shares of Stock which may be withheld with respect to the issuance, vesting, exercise or payment of any Award (or which may be
repurchased from the Participant of such Award within six months (or such other period as may be determined by the Administrator) after such shares of Stock were acquired by the Participant from the Company) in order to satisfy the
Participant’s federal, state, local and foreign tax liabilities with respect to the issuance, vesting, exercise or payment of the Award shall be limited to the number of shares which have a Fair Market Value on the date of withholding or
repurchase equal to the aggregate amount of such liabilities based on the minimum statutory withholding rates for federal, state, local and foreign income tax and employment tax purposes that are applicable to such supplemental taxable income. 

15.4 No Right to Employment or Services. Nothing in the Plan or any Award Agreement shall interfere with or limit in any way the right
of the Company or any Parent or Subsidiary to terminate any Participant’s employment or services at any time, nor confer upon any Participant any right to continue in the employ or service of the Company or any Parent or Subsidiary. 

15.5 Unfunded Status of Awards. The Plan is intended to be an “unfunded” plan for incentive compensation. With respect to any
payments not yet made to a Participant pursuant to an Award, nothing contained in the Plan or any Award Agreement shall give the Participant any rights that are greater than those of a general creditor of the Company or any Parent or Subsidiary.

 15.6 Indemnification. To the extent allowable pursuant to applicable law, the Administrator (and each member thereof) shall be
indemnified and held harmless by the Company from any loss, cost, liability, or expense that may be imposed upon or reasonably incurred by such member in connection with or resulting from any claim, action, suit, or proceeding to which he or she may
be a party or in which he or she may be involved by reason of any action or failure to act pursuant to the Plan and against and from any and all amounts paid by him or her in satisfaction of judgment in such action, suit, or proceeding against him
or her; provided he or she gives the Company an opportunity, at its own expense, to handle and defend the same before he or she undertakes to handle and defend it on his or her own behalf. The foregoing right of indemnification shall not be
exclusive of any other rights of indemnification to which such persons may be entitled pursuant to the Company’s Certificate of Incorporation or Bylaws, as a matter of law, or otherwise, or any power that the Company may have to indemnify them
or hold them harmless. 
 15.7 Relationship to Other Benefits. No payment pursuant to the Plan shall be taken into account in
determining any benefits pursuant to any pension, retirement, savings, profit sharing, group insurance, welfare or other benefit plan of the Company or any Parent or Subsidiary except to the extent otherwise expressly provided in writing in such
other plan or an agreement thereunder. 

  
 23 

 15.8 Expenses. The expenses of administering the Plan shall be borne by the Company and
its Parents and Subsidiaries. 
 15.9 Titles and Headings. The titles and headings of the Sections in the Plan are for convenience of
reference only and, in the event of any conflict, the text of the Plan, rather than such titles or headings, shall control. 
 15.10
Fractional Shares. No fractional shares of Stock shall be issued and the Administrator shall determine, in its discretion, whether cash shall be given in lieu of fractional shares or whether such fractional shares shall be eliminated by
rounding up or down as appropriate. 
 15.11 Limitations Applicable to Section 16 Persons. Notwithstanding any other provision
of the Plan, the Plan, and any Award granted or awarded to any Participant who is then subject to Section 16 of the Exchange Act, shall be subject to any additional limitations set forth in any applicable exemptive rule under Section 16 of
the Exchange Act (including any amendment to Rule 16b-3 under the Exchange Act) that are requirements for the application of such exemptive rule. To the extent permitted by applicable law, the Plan and Awards granted or awarded hereunder shall be
deemed amended to the extent necessary to conform to such applicable exemptive rule. 
 15.12 Government and Other Regulations. The
obligation of the Company to make payment of awards in Stock or otherwise shall be subject to all applicable laws, rules, and regulations, and to such approvals by government agencies as may be required. The Company shall be under no obligation to
register pursuant to the Securities Act any of the shares of Stock paid pursuant to the Plan. If the shares paid pursuant to the Plan may in certain circumstances be exempt from registration pursuant to the Securities Act, the Company may restrict
the transfer of such shares in such manner as it deems advisable to ensure the availability of any such exemption. 
 15.13 Governing
Law. The Plan and all Award Agreements shall be construed in accordance with and governed by the laws of the State of California, without regard to the conflicts of law principles thereof. 

15.14 Compliance with California Securities Laws. Unless determined otherwise by the Administrator, prior to the Public Trading Date,
this Plan is intended to comply with Section 25102(o) of the California Corporations Code and the regulations issued thereunder. Appendix I to the Plan sets forth the requirements under Section 25102(o) of the California Corporations Code
and the regulations issued thereunder and is incorporated herein by reference. If any of the provisions contained in this Plan are inconsistent with such requirements or Appendix I, such provisions shall be deemed null and void. The invalidity of
any provision of this Plan shall not affect the validity or enforceability of any other provision of this Plan, which shall remain in full force and effect. 

15.15 Appendices. The Board may approve such supplements to, or amendments, or appendices to, the Plan as it may consider
necessary or appropriate for purposes of compliance with applicable laws or otherwise and such supplements, amendments or appendices shall be considered a part of the Plan; provided, however, that no such supplements, amendments or appendices
shall increase the share limitation contained in Section 3.1 of the Plan. 

  
 24 

 APPENDIX I TO 

ULTRAGENYX PHARMACEUTICAL, INC. 

2011 EQUITY INCENTIVE PLAN 

California State Securities Law Compliance 

Notwithstanding anything to the contrary contained in the Plan and except as otherwise determined by the Administrator, the
provisions set forth in this Appendix shall apply to all Awards granted under the Ultragenyx Pharmaceutical, Inc. 2011 Equity Incentive Plan (the “Plan”) prior to the Public Trading Date. This Appendix shall be of no further
force and effect on or after the Public Trading Date. Definitions as set out in Article 2 of the Plan are applicable to this Appendix. 

The purpose of this Appendix is to set forth those provisions of the Plan necessary to comply with Section 25102(o) of the California
Corporations Code and the regulations issued thereunder. If any of the provisions contained in this Appendix are inconsistent with such requirements, such provisions shall be deemed null and void. The invalidity of any provision of this Appendix
shall not affect the validity or enforceability of any other provision of this Appendix, which shall remain in full force and effect. 

References to Articles and Sections set forth in this Appendix are to those Articles and Sections of the Plan. 

1.1 Term of Awards. The term of each Award shall be no more than ten years from the date of grant thereof. 

2.1 Award Exercise or Purchase Price. Except as provided in Article 11, the per share exercise or purchase price for the Stock to be
issued upon exercise of an Award shall be such price as is determined by the Administrator, but in the case of an Award granted to a Participant who, at the time of grant of such Award, owns stock representing more than 10% of the voting power of
all classes of stock of the Company or any parent (as defined in Section 175 of the California Corporations Code) or Subsidiary, the per share exercise or purchase price shall be no less than 110% of the Fair Market Value per share on the date
of the grant (100% in the case of an Award other than an Option). Notwithstanding the foregoing, Awards may be granted with a per share exercise or purchase price other than as required above pursuant to a merger or other corporate transaction. 

3.1 Exercisability. Except with regard to Awards granted to officers, members of the Board, managers or consultants, in no event shall
an Award granted hereunder become vested and exercisable at a rate of less than 20% per year over five years from the date the Award is granted, subject to reasonable conditions, such as continuing to be a member of the Board, Employee or
Consultant. 

  
 25 

 4.1 Exercisability Following Termination. 

(a) Termination Other Than Death or Disability. If a Participant has a Termination of Service for any reason other than
by reason of the Participant’s Disability or death, such Participant may exercise his or her Award within such period of time as is specified in the Award Agreement to the extent that the Award is vested on the date of termination; provided,
however, that prior to the Public Trading Date, such period of time shall not be less than thirty days (but in no event later than the expiration of the term of the Award as set forth in the Award Agreement). In the absence of a specified time
in the Award Agreement, the Option shall remain exercisable for three months following the Participant’s Termination of Service for any reason other than death or Disability. 

(b) Death. If a Participant has a Termination of Service as a result of the Participant’s death, the Award may be
exercised within such period of time as is specified in the Award Agreement; provided, however, that prior to the Public Trading Date, such period of time shall not be less than six months (but in no event later than the expiration of the
term of such Award as set forth in the Notice of Grant), by the Participant’s estate or by a person who acquires the right to exercise the Award by bequest or inheritance, but only to the extent that the Award is vested on the date of death. In
the absence of a specified time in the Award Agreement, the Award shall remain exercisable for twelve months following the Participant’s Termination of Service for death. 

(c) Disability of Participant. If a Participant has a Termination of Service as a result of the Participant’s
Disability, the Participant may exercise his or her Award within such period of time as is specified in the Award Agreement to the extent the Award is vested on the date of termination; provided, however, that prior to the Public Trading
Date, such period of time shall not be less than six months (but in no event later than the expiration of the term of such Award as set forth in the Award Agreement). In the absence of a specified time in the Award Agreement, the Award shall remain
exercisable for twelve months following the Participant’s Termination of Service for Disability. 
 (d)
Misconduct of Participant. If a Participant has a Termination of Service as a result of the Participant’s Misconduct, the Award shall terminate immediately and cease to remain outstanding. 

5.1 Repurchase Provisions. In the event the Administrator provides that the Company may repurchase Stock acquired upon exercise of an
Award upon the occurrence of certain specified events, including, without limitation, a Participant’s Termination of Service, divorce, bankruptcy or insolvency, then any such repurchase right shall be set forth in the applicable Award Agreement
or in another agreement referred to in such agreement and, to the extent required by Section 260.140.41 and Section 260.140.42 of Title 10 of the California Code of Regulations (or any successor regulation), any such repurchase right set
forth in an Award granted prior to the Public Trading Date to a person who is not an officer, member of the Board, manager or consultant shall be upon the following terms: (i) if the repurchase option gives the Company the right to repurchase
the shares upon the Participant’s Termination of Service at not less than the Fair Market Value of the shares to be purchased on the date of termination of employment or service, then (A) the right to repurchase shall be exercised for cash
or 

  
 26 

 
cancellation of purchase money indebtedness for the shares within ninety days of termination (or in the case of shares issued upon exercise of Awards after such date of termination, within ninety
days after the date of the exercise) or such longer period as may be agreed to by the Administrator and the Participant and (B) the right terminates on the Public Trading Date; and (ii) if the repurchase option gives the Company the right
to repurchase the Stock upon the Participant’s Termination of Service at the original purchase price for such Stock, then (A) the right to repurchase at the original purchase price shall lapse at the rate of at least 20% of the shares per
year over five (5) years from the date the Award is granted (without respect to the date the Award was exercised or became exercisable) and (B) the right to repurchase shall be exercised for cash or cancellation of purchase money
indebtedness for the shares within ninety days of termination (or, in the case of shares issued upon exercise of Awards, after such date of termination, within ninety days after the date of the exercise) or such longer period as may be agreed to by
the Company and the Participant. 
 6.1 Information Rights. Prior to the Public Trading Date and to the extent required by
Section 260.140.46 of Title 10 of the California Code of Regulations, the Company shall provide to each Participant and to each individual who acquires Stock pursuant to the Plan, not less frequently than annually during the period such
Participant has one or more Awards outstanding, and, in the case of an individual who acquires Stock pursuant to the Plan, during the period such individual owns such Stock, copies of annual financial statements. Notwithstanding the preceding
sentence, the Company shall not be required to provide such statements to key employees whose duties in connection with the Company assure their access to equivalent information. 

7.1 Transferability. Prior to the Public Trading Date, no Award shall be assigned, transferred, or otherwise disposed of by a
Participant other than by will or the laws of descent and distribution or, with respect to Awards other than Incentive Stock Options, as permitted by Rule 701 of the Securities Act. 

8.1 Limitation on Number of Shares. Prior to the Public Trading Date, at no time shall the total number of shares of Stock issuable
upon exercise of all outstanding Options under the Plan and any shares of Stock provided for under any bonus or similar plan or agreement of the Company exceed 30% of the then-outstanding shares of Stock of the Company, as calculated pursuant to
Section 260.140.45 of Title 10 of the California Code of Regulations (or any successor regulation), unless a percentage higher than 30% is approved by at least two-thirds of the outstanding securities of the Company entitled to vote. The number
of shares of Stock which may be issued or transferred pursuant to Awards under the Plan shall be reduced to the extent necessary to comply with this provision. 

  
 27 

 ULTRAGENYX PHARMACEUTICAL, INC. 

2011 EQUITY INCENTIVE PLAN STOCK OPTION GRANT NOTICE AND 

STOCK OPTION AGREEMENT 

Ultragenyx Pharmaceutical, Inc. (the “Company”), pursuant to its 2011 Equity Incentive Plan (the
“Plan”), hereby grants to the holder listed below (“Participant”), an option to purchase the number of shares of the Company’s Stock set forth below (the “Option”). This
Option is subject to all of the terms and conditions as set forth herein and in the Stock Option Agreement attached hereto as Exhibit A (the “Stock Option Agreement”) and the Plan, each of which are incorporated herein
by reference. Unless otherwise defined herein, the terms defined in the Plan shall have the same defined meanings in this Grant Notice and the Stock Option Agreement. 
  

							
	 Participant:
	 		 	 
			
	 Grant Date:
	 		 	 
			
	 Vesting Commencement Date:
	 		 	 
				
	 Exercise Price per Share:
	 		 	$  	  	 
				
	 Total Exercise Price:
	 		 	$  	  	 
			
	 Total Number of Shares Subject to the Option:
	 		 	 
			
	 Expiration Date:
	 		 	 

 Type of Option:   ̈
        Incentive Stock Option                  ̈  Non-Qualified Stock
Option 
 Vesting Schedule: [To be specified in individual agreements.] 

By his or her signature and the Company’s signature below, Participant agrees to be bound by the terms and conditions of the Plan, the
Stock Option Agreement and this Grant Notice. Participant has reviewed the Stock Option Agreement, the Plan and this Grant Notice in their entirety, has had an opportunity to obtain the advice of counsel prior to executing this Grant Notice and
fully understands all provisions of this Grant Notice, the Stock Option Agreement and the Plan. Participant hereby agrees to accept as binding, conclusive and final all decisions or interpretations of the Administrator of the Plan upon any questions
arising under the Plan or the Option. 
  

									
	ULTRAGENYX PHARMACEUTICAL, INC.	 		 	PARTICIPANT:
					
	By:	 	 	 		 	By:	 	 

									
					
	Print Name:	 		 		 	Print Name:	 	 

									
					
	 Title:
	 		 		 		 	
					
	 Address:
	 		 		 	 Address:
	 	 
					
		 		 		 		 	 

 EXHIBIT A 

TO STOCK OPTION GRANT NOTICE 

STOCK OPTION AGREEMENT 

Pursuant to the Stock Option Grant Notice (“Grant Notice”) to which this Stock Option Agreement (this
“Agreement”) is attached, Ultragenyx Pharmaceutical, Inc. (the “Company”) has granted to Participant an option under the Company’s 2011 Equity Incentive Plan (the “Plan”)
to purchase the number of shares of Stock indicated in the Grant Notice. 
 ARTICLE I 

GENERAL 
 1.1 Defined
Terms. Capitalized terms not specifically defined herein shall have the meanings specified in the Plan and the Grant Notice. 
 1.2
Incorporation of Terms of Plan. The Option is subject to the terms and conditions of the Plan which are incorporated herein by reference. 

ARTICLE II 
 GRANT OF
OPTION 
 2.1 Grant of Option. In consideration of Participant’s past and/or continued employment with or service to the
Company or a Parent or Subsidiary and for other good and valuable consideration, effective as of the Grant Date set forth in the Grant Notice (the “Grant Date”), the Company irrevocably grants to Participant the Option to
purchase any part or all of an aggregate of the number of shares of Stock set forth in the Grant Notice, upon the terms and conditions set forth in the Plan and this Agreement. Unless designated as a Non-Qualified Stock Option in the Grant Notice,
the Option shall be an Incentive Stock Option to the maximum extent permitted by law. 
 2.2 Exercise Price. The exercise
price of the shares of Stock subject to the Option shall be as set forth in the Grant Notice, without commission or other charge; provided, however, that if this Option is designated as an Incentive Stock Option, the price per share of the
shares subject to the Option shall not be less than the greater of (i) 100% of the Fair Market Value of a share of Stock on the Grant Date, or (ii) 110% of the Fair Market Value of a share of Stock on the Grant Date in the case of a
Participant then owning (within the meaning of Section 424(d) of the Code) more than 10% of the total combined voting power of all classes of stock of the Company or any “subsidiary corporation” of the Company or any “parent
corporation” of the Company (each within the meaning of Section 424 of the Code). 

  
 A-1 

 ARTICLE III 

PERIOD OF EXERCISABILITY 

3.1 Commencement of Exercisability. 

(a) Subject to Sections 3.3 and 5.8, the Option shall become vested and exercisable in such amounts and at such times as are
set forth in the Grant Notice. 
 (b) No portion of the Option which has not become vested and exercisable at the date of
Participant’s Termination of Service shall thereafter become vested and exercisable, except as may be otherwise provided by the Administrator or as set forth in a written agreement between the Company and Participant. 

3.2 Duration of Exercisability. The installments provided for in the vesting schedule set forth in the Grant Notice are cumulative.
Each such installment which becomes vested and exercisable pursuant to the vesting schedule set forth in the Grant Notice shall remain vested and exercisable until it becomes unexercisable under Section 3.3. 

3.3 Expiration of Option. The Option may not be exercised to any extent by anyone after the first to occur of the following events:

 (a) The expiration of ten years from the Grant Date; 

(b) If this Option is designated as an Incentive Stock Option and Participant owned (within the meaning of Section 424(d)
of the Code), at the time the Option was granted, more than 10% of the total combined voting power of all classes of stock of the Company or any “subsidiary corporation” of the Company or “parent corporation” of the Company (each
within the meaning of Section 424 of the Code), the expiration of five years from the date the Option was granted; or 

(c) The expiration of three months following the date of Participant’s Termination of Service, unless such termination
occurs by reason of Participant’s death, Disability or Misconduct; 
 (d) The expiration of one year following the date
of Participant’s Termination of Service by reason of Participant’s death or Disability; or 
 (e) The date of
Participant’s Termination of Service as a result of Participant’s Misconduct. 
 Participant acknowledges that an Incentive Stock
Option exercised more than three months after Participant’s termination of status as an Employee, other than by reason of death or Disability, will be taxed as a Non-Qualified Stock Option. 

3.4 Special Tax Consequences. Participant acknowledges that, to the extent that the aggregate Fair Market Value (determined as of the
time the Option is granted) of all shares of Stock with respect to which Incentive Stock Options, including the Option, are first exercisable for the first time by Participant in any calendar year exceeds $100,000 (or such other limitation

  
 A-2 

 
as imposed by Section 422(d) of the Code), the Option and such other options shall be treated as not qualifying under Section 422 of the Code but rather shall be considered
Non-Qualified Stock Options. Participant further acknowledges that the rule set forth in the preceding sentence shall be applied by taking Options and other “incentive stock options” into account in the order in which they were granted.

 ARTICLE IV 

EXERCISE OF OPTION 
 4.1
Person Eligible to Exercise. Except as provided in Sections 5.2(b) and 5.2(c), during the lifetime of Participant, only Participant may exercise the Option or any portion thereof. After the death of Participant, any exercisable portion of the
Option may, prior to the time when the Option becomes unexercisable under Section 3.3, be exercised by Participant’s personal representative or by any person empowered to do so under the deceased Participant’s will or under the then
applicable laws of descent and distribution. 
 4.2 Partial Exercise. Any exercisable portion of the Option or the entire Option, if
then wholly exercisable, may be exercised in whole or in part at any time prior to the time when the Option or portion thereof becomes unexercisable under Section 3.3. 

4.3 Manner of Exercise. The Option, or any exercisable portion thereof, may be exercised solely by delivery to the Secretary of the
Company or the Secretary’s office of all of the following prior to the time when the Option or such portion thereof becomes unexercisable under Section 3.3: 

(a) An Exercise Notice in writing signed by Participant or any other person then entitled to exercise the Option or portion
thereof, stating that the Option or portion thereof is thereby exercised, such notice complying with all applicable rules established by the Administrator. Such notice shall be substantially in the form attached as Exhibit B to the Grant
Notice (or such other form as is prescribed by the Administrator); and 
 (b) Subject to Section 5.1(c) of the Plan:

 (i) Full payment (in cash or by check) for the shares with respect to which the Option or portion thereof is exercised; or

 (ii) With the consent of the Administrator, by delivery of a full recourse promissory note on such terms and conditions as
may be approved by the Administrator; or 
 (iii) With the consent of the Administrator, by delivery of shares of Stock then
issuable upon exercise of the Option having a Fair Market Value on the date of delivery equal to the aggregate exercise price of the Option or exercised portion thereof; or 

(iv) On and after the Public Trading Date, such payment may be made, in whole or in part, through the delivery of shares of
Stock which have been owned by Participant for at least six months, duly endorsed for transfer to the Company with a Fair Market Value on the date of delivery equal to the aggregate exercise price of the Option or exercised portion thereof; or 

  
 A-3 

 (v) On and after the Public Trading Date, through the delivery of a notice
that Participant has placed a market sell order with a broker with respect to shares of Stock then issuable upon exercise of the Option, and that the broker has been directed to pay a sufficient portion of the net proceeds of the sale to the Company
in satisfaction of the Option exercise price; provided, that payment of such proceeds is made to the Company upon settlement of such sale; or 

(vi) Subject to any applicable laws, any combination of the consideration provided in the foregoing paragraphs (i),
(ii) and (iii); and 
 (c) A bona fide written representation and agreement, in such form as is prescribed by the
Administrator, signed by Participant or the other person then entitled to exercise such Option or portion thereof, stating that the shares of Stock are being acquired for Participant’s own account, for investment and without any present
intention of distributing or reselling said shares or any of them except as may be permitted under the Securities Act and then applicable rules and regulations thereunder, and that Participant or other person then entitled to exercise such Option or
portion thereof will indemnify the Company against and hold it free and harmless from any loss, damage, expense or liability resulting to the Company if any sale or distribution of the shares by such person is contrary to the representation and
agreement referred to above. The Administrator may, in its absolute discretion, take whatever additional actions it deems appropriate to ensure the observance and performance of such representation and agreement and to effect compliance with the
Securities Act and any other federal or state securities laws or regulations. Without limiting the generality of the foregoing, the Administrator may require an opinion of counsel acceptable to it to the effect that any subsequent transfer of shares
acquired on an Option exercise does not violate the Securities Act, and may issue stop-transfer orders covering such shares. Share certificates evidencing Stock issued on exercise of the Option shall bear an appropriate legend referring to the
provisions of this subsection (c) and the agreements herein. The written representation and agreement referred to in the first sentence of this subsection (c) shall, however, not be required if the shares to be issued pursuant to such
exercise have been registered under the Securities Act, and such registration is then effective in respect of such shares; and 

(d) The receipt by the Company of full payment for such shares, including payment of any applicable withholding tax, which may
be in the form of consideration used by Participant to pay for such shares under Section 4.3(b), subject to Section 15.3 of the Plan; and 

(e) In the event the Option or portion thereof shall be exercised pursuant to Section 4.1 by any person or persons other
than Participant, appropriate proof of the right of such person or persons to exercise the Option. 
 4.4 Conditions to Issuance of Stock
Certificates. The shares of Stock deliverable upon the exercise of the Option, or any portion thereof, may be either previously authorized but unissued shares or issued shares which have then been reacquired by the Company. Such shares shall be
fully paid and nonassessable. The Company shall not be required to issue or deliver any shares of Stock purchased upon the exercise of the Option or portion thereof prior to fulfillment of all of the following conditions: 

(a) The admission of such shares to listing on all stock exchanges on which such Stock is then listed; and 

  
 A-4 

 (b) The completion of any registration or other qualification of such shares
under any state or federal law or under rulings or regulations of the Securities and Exchange Commission or of any other governmental regulatory body, which the Administrator shall, in its absolute discretion, deem necessary or advisable; and 

(c) The obtaining of any approval or other clearance from any state or federal governmental agency which the Administrator
shall, in its absolute discretion, determine to be necessary or advisable; and 
 (d) The lapse of such reasonable period of
time following the exercise of the Option as the Administrator may from time to time establish for reasons of administrative convenience; and 

(e) The receipt by the Company of full payment for such shares, including payment of any applicable withholding tax, which may
be in the form of consideration used by Participant to pay for such shares under Section 4.3(b), subject to Section 15.3 of the Plan. 

4.5 Rights as Stockholder. The holder of the Option shall not be, nor have any of the rights or privileges of, a stockholder of the
Company in respect of any shares purchasable upon the exercise of any part of the Option unless and until such shares shall have been issued by the Company to such holder. 

ARTICLE V 
 OTHER
PROVISIONS 
 5.1 Administration. The Administrator shall have the power to interpret the Plan and this Agreement and to adopt
such rules for the administration, interpretation and application of the Plan as are consistent therewith and to interpret, amend or revoke any such rules. All actions taken and all interpretations and determinations made by the Administrator in
good faith shall be final and binding upon Participant, the Company and all other interested persons. No member of the Administrator shall be personally liable for any action, determination or interpretation made in good faith with respect to the
Plan, this Agreement or the Option. In its absolute discretion, the Board may at any time and from time to time exercise any and all rights and duties of the Administrator under the Plan and this Agreement. 

5.2 Option Not Transferable. 

(a) Subject to Section 5.2(b), the Option may not be sold, pledged, assigned or transferred in any manner other than by
will or the laws of descent and distribution, unless and until the shares underlying the Option have been issued, and all restrictions applicable to such shares have lapsed. Neither the Option nor any interest or right therein shall be liable for
the debts, contracts or engagements of Participant or his or her successors in interest or shall be 

  
 A-5 

 
subject to disposition by transfer, alienation, anticipation, pledge, encumbrance, assignment or any other means whether such disposition be voluntary or involuntary or by operation of law by
judgment, levy, attachment, garnishment or any other legal or equitable proceedings (including bankruptcy), and any attempted disposition thereof shall be null and void and of no effect, except to the extent that such disposition is permitted by the
preceding sentence. 
 (b) Notwithstanding any other provision in this Agreement, with the consent of the Administrator and
to the extent the Option is designated as a Non-Qualified Stock Option, the Option may be transferred to, exercised by and paid to one or more Permitted Transferees, subject to the terms and conditions set forth in Section 10.3 of the Plan.

 (c) Unless transferred to a Permitted Transferee in accordance with Section 5.2(b), during the lifetime of
Participant, only Participant may exercise the Option or any portion thereof. Subject to such conditions and procedures as the Administrator may require, a Permitted Transferee may exercise the Option or any portion thereof during Participant’s
lifetime. After the death of Participant, any exercisable portion of the Option may, prior to the time when the Option becomes unexercisable under Section 3.3, be exercised by Participant’s personal representative or by any person
empowered to do so under the deceased Participant’s will or under the then applicable laws of descent and distribution. 

5.3 Lock-Up Period. Participant hereby agrees that, if so requested by the Company or any representative of the
underwriters (the “Managing Underwriter”) in connection with any registration of the offering of any securities of the Company under the Securities Act, Participant shall not sell or otherwise transfer any shares of Stock or
other securities of the Company during such period as may be requested in writing by the Managing Underwriter and agreed to in writing by the Company (which period shall not be longer than one hundred eighty days) (the “Market Standoff
Period”) following the effective date of a registration statement of the Company filed under the Securities Act; provided, however, that such restriction shall apply only to the first registration
statement of the Company to become effective under the Securities Act that includes securities to be sold on behalf of the Company to the public in an underwritten public offering under the Securities Act. 

5.4 Restrictive Legends and Stop-Transfer Orders. 

(a) The share certificate or certificates evidencing the shares of Stock purchased hereunder shall be endorsed with any legends
that may be required by state or federal securities laws. 
 (b) Participant agrees that, in order to ensure compliance with
the restrictions referred to herein, the Company may issue appropriate “stop transfer” instructions to its transfer agent, if any, and that, if the Company transfers its own securities, it may make appropriate notations to the same effect
in its own records. 
 (c) The Company shall not be required: (i) to transfer on its books any shares of Stock that have
been sold or otherwise transferred in violation of any of the provisions of this Agreement, or (ii) to treat as owner of such shares of Stock or to accord the right to vote or pay dividends to any purchaser or other transferee to whom such
shares shall have been so transferred. 

  
 A-6 

 5.5 Shares to Be Reserved. The Company shall at all times during the term of the Option
reserve and keep available such number of shares of Stock as will be sufficient to satisfy the requirements of this Agreement. 
 5.6
Notices. Any notice to be given under the terms of this Agreement to the Company shall be addressed to the Company in care of the Secretary of the Company at the address given beneath the signature of the Company’s authorized officer on
the Grant Notice, and any notice to be given to Participant shall be addressed to Participant at the address given beneath Participant’s signature on the Grant Notice. By a notice given pursuant to this Section 5.6, either party may
hereafter designate a different address for notices to be given to that party. Any notice which is required to be given to Participant shall, if Participant is then deceased, be given to the person entitled to exercise his or her Option pursuant to
Section 4.1 by written notice under this Section 5.6. Any notice shall be deemed duly given when sent via email or when sent by certified mail (return receipt requested) and deposited (with postage prepaid) in a post office or branch post
office regularly maintained by the United States Postal Service. 
 5.7 Titles. Titles are provided herein for convenience only and
are not to serve as a basis for interpretation or construction of this Agreement. 
 5.8 Stockholder Approval. The Plan will be
submitted for approval by the Company’s stockholders within twelve months after the date the Plan was initially adopted by the Board. The Option may not be exercised to any extent by anyone prior to the time when the Plan is approved by the
stockholders, and if such approval has not been obtained by the end of said twelve month period, the Option shall thereupon be canceled and become null and void. 

5.9 Governing Law; Severability. This Agreement shall be administered, interpreted and enforced under the laws of the State of
California, without regard to the conflicts of law principles thereof. Should any provision of this Agreement be determined by a court of law to be illegal or unenforceable, the other provisions shall nevertheless remain effective and shall remain
enforceable. 
 5.10 Conformity to Securities Laws. Participant acknowledges that the Plan is intended to conform to the extent
necessary with all provisions of the Securities Act and the Exchange Act and any and all regulations and rules promulgated by the Securities and Exchange Commission thereunder, and state securities laws and regulations. Notwithstanding anything
herein to the contrary, the Plan shall be administered, and the Option is granted and may be exercised, only in such a manner as to conform to such laws, rules and regulations. To the extent permitted by applicable law, the Plan and this Agreement
shall be deemed amended to the extent necessary to conform to such laws, rules and regulations. 
 5.11 Amendments. This Agreement
may not be modified, amended or terminated except by an instrument in writing, signed by Participant or such other person as may be permitted to exercise the Option pursuant to Section 4.1 and by a duly authorized representative of the Company.

  
 A-7 

 5.12 No Employment Rights. If Participant is an Employee, nothing in the Plan or this
Agreement shall confer upon Participant any right to continue in the employ of the Company or any Subsidiary or shall interfere with or restrict in any way the rights of the Company and its Subsidiaries, which are expressly reserved, to discharge
Participant at any time for any reason whatsoever, with or without cause, except to the extent expressly provided otherwise in a written agreement between the Company and Participant. 

5.13 Successors and Assigns. The Company may assign any of its rights under this Agreement to single or multiple assignees, and this
Agreement shall inure to the benefit of the successors and assigns of the Company. Subject to the restrictions on transfer herein set forth, this Agreement shall be binding upon Participant and his or her heirs, executors, administrators, successors
and assigns. 
 5.14 Notification of Disposition. If this Option is designated as an Incentive Stock Option, Participant shall give
prompt notice to the Company of any disposition or other transfer of any shares of Stock acquired under this Agreement if such disposition or transfer is made (a) within two years from the Grant Date with respect to such shares or
(b) within one year after the transfer of such shares to him. Such notice shall specify the date of such disposition or other transfer and the amount realized, in cash, other property, assumption of indebtedness or other consideration, by
Participant in such disposition or other transfer. 
 5.15 Limitations Applicable to Section 16 Persons. Notwithstanding any
other provision of the Plan or this Agreement, if Participant is subject to Section 16 of the Exchange Act, the Plan, the Option and this Agreement shall be subject to any additional limitations set forth in any applicable exemptive rule under
Section 16 of the Exchange Act (including any amendment to Rule 16b-3 of the Exchange Act) that are requirements for the application of such exemptive rule. To the extent permitted by applicable law, this Agreement shall be deemed amended to
the extent necessary to conform to such applicable exemptive rule. 
 5.16 Entire Agreement. The Plan and this Agreement (including
all Exhibits hereto) constitute the entire agreement of the parties and supersede in their entirety all prior undertakings and agreements of the Company and Participant with respect to the subject matter hereof. 

  
 A-8 

 EXHIBIT B 

TO STOCK OPTION GRANT NOTICE 

FORM OF EXERCISE NOTICE 

Effective as of today,         ,
                 the undersigned (“Participant”) hereby elects to exercise
                 Participant’s option to purchase shares of the Stock (the “Shares”) of Ultragenyx Pharmaceutical, Inc. (the
“Company”) under and pursuant to the Ultragenyx Pharmaceutical, Inc. 2011 Equity Incentive Plan (the “Plan”) and the Stock Option Grant Notice and Stock Option Agreement dated
            ,          (the “Option Agreement”). Capitalized terms used herein without definition shall have
the meanings given in the Option Agreement. 
  

							
	 Grant Date:
	 		 	 
			
	 Number of Shares as to which Option is Exercised:
	 		 	 
				
	 Exercise Price per Share:
	 		 	 $  
	  	 
				
	 Total Exercise Price:
	 		 	 $  
	  	 
			
	 Certificate to the issued in the name of:
	 		 	 
			
	 Cash Payment delivered herewith:
	 		 	$ __________________ (Representing the full Exercise Price for the Shares, as well as any applicable withholding tax)

 Type of
Option:   ̈      Incentive Stock Option                  ̈  Non-Qualified Stock Option 
 1. Representations of Participant. Participant
acknowledges that Participant has received, read and understood the Plan and the Option Agreement. Participant agrees to abide by and be bound by their terms and conditions. 

2. Rights as Stockholder. Until the Shares are issued (as evidenced by the appropriate entry on the books of the Company or of a duly
authorized transfer agent of the Company), no right to vote or receive dividends or any other rights as a stockholder shall exist with respect to Shares subject to the Option, notwithstanding the exercise of the Option. No adjustment will be made
for a dividend or other right for which the record date is prior to the date the Shares are issued, except as provided in Article 10 of the Plan. 

Participant shall enjoy rights as a stockholder until such time as Participant disposes of the Shares or the Company and/or its assignee(s)
exercises the Right of First Refusal hereunder. Upon such exercise, Participant shall have no further rights as a holder of the Shares so purchased except the right to receive payment for the Shares so purchased in accordance with the provisions of
this Agreement, and Participant shall forthwith cause the certificate(s), if any issued, evidencing the Shares so purchased to be surrendered to the Company for transfer or cancellation. 

  
 B-1 

 3. Participant’s Rights to Transfer Shares. 

(a) Before any Shares held by Participant or any permitted transferee (each, a “Holder”)
may be sold, pledged, assigned, hypothecated, transferred, or otherwise disposed of (each, a “Transfer”), the Company or its assignee(s) shall have a right of first refusal to purchase the Shares proposed to be Transferred on
the terms and conditions set forth in this Section (the “Right of First Refusal”). In the event that the Company’s Bylaws contain a right of first refusal with respect to the Shares, such right of first refusal shall
apply to the Shares to the extent such provisions are more restrictive than the Right of First Refusal set forth in this Section and the Right of First Refusal set forth in this Section shall not in any way restrict the operation of the
Company’s Bylaws. 
 (b) In the event any Holder desires to Transfer any Shares, the Holder
shall deliver to the Company a written notice (the “Notice”) stating: (i) the Holder’s bona fide intention to sell or otherwise Transfer such Shares; (ii) the name of each proposed purchaser or other transferee
(“Proposed Transferee”); (iii) the number of Shares to be Transferred to each Proposed Transferee; and (iv) the price for which the Holder proposes to Transfer the Shares (the “Offered
Price”), and the Holder shall offer such Shares at the Offered Price to the Company or its assignee(s). 

(c) Within twenty-five days after receipt of the Notice, the Company and/or its assignee(s) may elect in writing
to purchase all, but not less than all, of the Shares proposed to be Transferred to any one or more of the Proposed Transferees by delivery of a written exercise notice to the Holder (a “Company Notice”). The purchase price
will be determined in accordance with subsection (d) below. 
 (d) The purchase price
(“Purchase Price”) for the Shares repurchased under this Section shall be the Offered Price. 

(e) Payment of the Purchase Price shall be made, at the option of the Company or its assignee(s), in cash (by check), by
cancellation of all or a portion of any outstanding indebtedness of the Holder to the Company (or, in the case of repurchase by an assignee, to the assignee), or by any combination thereof within five days after delivery of the Company Notice or in
the manner and at the times mutually agreed to by the Company and the Holder. Should the Offered Price specified in the Notice be payable in property other than cash, the Company shall have the right to pay the purchase price in the form of cash
equal in amount to the value of such property. If the Holder and the Company cannot agree on such cash value within ten days after the Company’s receipt of the Notice, the valuation shall be made by the Board. The payment of the purchase price
shall then be held on the later of (i) five days following delivery of the Company Notice or (ii) five days after such valuation shall have been made. 

(f) If all or a portion of the Shares proposed in the Notice to be Transferred are not purchased by the Company and/or its
assignee(s) as provided in this Section, then the Holder may sell or otherwise Transfer such Shares to that Proposed Transferee at the Offered Price or at a higher price, provided that such sale or other Transfer is consummated within sixty days
after the date of the Notice and provided further that any such sale or other Transfer is 

  
 B-2 

 
effected in accordance with any applicable securities laws and the Proposed Transferee agrees in writing that the provisions of this Section shall continue to apply to the Shares in the hands of
such Proposed Transferee. If the Shares described in the Notice are not Transferred to the Proposed Transferee within such sixty-day period, a new Notice shall be given to the Company, and the Company and/or its assignees shall again be offered the
Right of First Refusal as provided herein before any Shares held by the Holder may be sold or otherwise Transferred. 

(g) Anything to the contrary contained in this Section notwithstanding, the Transfer of any or all of the Shares
during Participant’s lifetime or upon Participant’s death by will or intestacy to Participant’s Immediate Family or a trust for the benefit of Participant’s Immediate Family shall be exempt from the Right of First Refusal. As
used herein, “Immediate Family” shall mean spouse, lineal descendant or antecedent, father, mother, brother or sister or stepchild (whether or not adopted). In such case, the transferee or other recipient shall receive and
hold the Shares so Transferred subject to the provisions of this Section (including the Right of First Refusal) and the Restricted Stock Purchase Agreement, if applicable, and there shall be no further Transfer of such Shares except in accordance
with the terms of this Section. 
 (h) The Right of First Refusal shall terminate as to all Shares upon the
Public Trading Date. 
 (i) Any transfer or sale of the Shares is subject to restrictions on transfer imposed by any
applicable state and federal securities laws. Any Transfer or attempted Transfer of any of the Shares not in accordance with the terms of this Agreement shall be void and the Company may enforce the terms of this Agreement by stop transfer
instructions or similar actions by the Company and its agents or designees. 
 4. Tax Consultation. Participant understands that
Participant may suffer adverse tax consequences as a result of Participant’s purchase or disposition of the Shares. Participant represents that Participant has consulted with any tax consultants Participant deems advisable in connection with
the purchase or disposition of the Shares and that Participant is not relying on the Company for any tax advice. 
 5. Restrictive
Legends and Stop-Transfer Orders. 
 (a) Legends. Participant understands and agrees that the Company shall cause
any certificates issued evidencing the Shares shall have the legends set forth below or legends substantially equivalent thereto, together with any other legends that may be required by state or federal securities laws: 

THE SHARES REPRESENTED HEREBY HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED (“ACT”), NOR HAVE THEY BEEN
REGISTERED OR QUALIFIED UNDER THE SECURITIES LAWS OF ANY STATE. NO TRANSFER OF SUCH SECURITIES WILL BE PERMITTED UNLESS A REGISTRATION STATEMENT UNDER THE ACT IS IN EFFECT AS TO SUCH TRANSFER, THE TRANSFER IS MADE IN ACCORDANCE WITH RULE 144 UNDER
THE ACT, OR IN THE 

  
 B-3 

 
OPINION OF COUNSEL (WHICH MAY BE COUNSEL FOR THE COMPANY) REGISTRATION UNDER THE ACT IS UNNECESSARY IN ORDER FOR SUCH TRANSFER TO COMPLY WITH THE ACT AND WITH APPLICABLE STATE SECURITIES LAWS.

 THE SHARES REPRESENTED BY THIS CERTIFICATE MAY BE TRANSFERRED ONLY IN ACCORDANCE WITH THE TERMS OF AN AGREEMENT BETWEEN THE COMPANY AND
THE STOCKHOLDER, A COPY OF WHICH IS ON FILE WITH THE SECRETARY OF THE COMPANY. SUCH TRANSFER RESTRICTIONS ARE BINDING ON TRANSFEREES OF THESE SHARES. 

(b) Participant agrees that, in order to ensure compliance with the restrictions referred to herein, the Company may issue
appropriate “stop transfer” instructions to its transfer agent, if any, and that, if the Company transfers its own securities, it may make appropriate notations to the same effect in its own records. 

(c) The Company shall not be required (i) to transfer on its books any Shares that have been sold or otherwise transferred
in violation of any of the provisions of this Agreement or (ii) to treat as owner of such Shares or to accord the right to vote or pay dividends to any purchaser or other transferee to whom such Shares shall have been so transferred. 

6. Participant Representations. Participant hereby makes the following certifications and representations with respect to the Shares
listed above: 
 (a) Participant is aware of the Company’s business affairs and financial condition and has acquired
sufficient information about the Company to reach an informed and knowledgeable decision to acquire the Shares. Participant is acquiring these Shares for investment for Participant’s own account only and not with a view to, or for resale in
connection with, any “distribution” thereof within the meaning of the Securities Act. 
 (b) Participant
acknowledges and understands that the Shares constitute “restricted securities” under the Securities Act and have not been registered under the Securities Act in reliance upon a specific exemption therefrom, which exemption depends upon,
among other things, the bona fide nature of Participant’s investment intent as expressed herein. Participant understands that the Shares must be held indefinitely unless they are subsequently registered under the Securities Act or an exemption
from such registration is available. Participant further acknowledges and understands that the Company is under no obligation to register the Shares. Participant understands that the certificate evidencing the Shares will be imprinted with a legend
which prohibits the transfer of the Shares unless they are registered or such registration is not required in the opinion of counsel satisfactory to the Company and any other legend required under applicable state securities laws. 

(c) Participant is familiar with the provisions of Rule 701 and Rule 144, each promulgated under the Securities Act, which, in
substance, permit limited public resale of “restricted securities” acquired, directly or indirectly from the issuer thereof, in a non-public 

  
 B-4 

 
offering subject to the satisfaction of certain conditions. Rule 701 provides that if the issuer qualifies under Rule 701 at the time of the grant of the Option to Participant, the exercise will
be exempt from registration under the Securities Act. In the event the Company becomes subject to the reporting requirements of Section 13 or 15(d) of the Exchange Act, ninety days thereafter (or such longer period as any market stand-off
agreement may require) the securities exempt under Rule 701 may be resold, subject to the satisfaction of certain of the conditions specified by Rule 144, including: (i) the resale being made through a broker in an unsolicited
“broker’s transaction” or in transactions directly with a market maker (as said term is defined under the Exchange Act); and, in the case of an affiliate, (ii) the availability of certain public information about the Company,
(iii) the amount of securities being sold during any three month period not exceeding the limitations specified in Rule 144(e), and (iv) the timely filing of a Form 144, if applicable. 

(d) In the event that the Company does not qualify under Rule 701 at the time of grant of the Option, then the securities may
be resold in certain limited circumstances subject to the provisions of Rule 144, which requires the resale to occur not less than one year after the later of the date the securities were sold by the Company or the date the securities were sold by
an affiliate of the Company, within the meaning of Rule 144; and, in the case of acquisition of the securities by an affiliate, or by a non-affiliate who subsequently holds the securities less than two years, the satisfaction of the conditions set
forth in sections (i), (ii), (iii) and (iv) of paragraph (c) above. 
 (e) Participant further understands
that in the event all of the applicable requirements of Rule 701 or 144 are not satisfied, registration under the Securities Act, compliance with Regulation A, or some other registration exemption will be required; and that, notwithstanding the fact
that Rules 144 and 701 are not exclusive, the Staff of the Securities and Exchange Commission has expressed its opinion that persons proposing to sell private placement securities other than in a registered offering and otherwise than pursuant to
Rules 144 or 701 will have a substantial burden of proof in establishing that an exemption from registration is available for such offers or sales, and that such persons and their respective brokers who participate in such transactions do so at
their own risk. Participant understands that no assurances can be given that any such other registration exemption will be available in such event. 

7. Successors and Assigns. The Company may assign any of its rights under this Agreement to single or multiple assignees, and this
Agreement shall inure to the benefit of the successors and assigns of the Company. Subject to the restrictions on transfer herein set forth, this Agreement shall be binding upon Participant and his or her heirs, executors, administrators, successors
and assigns. 
 8. Interpretation. Any dispute regarding the interpretation of this Agreement shall be submitted by Participant or by
the Company forthwith to the Administrator, which shall review such dispute at its next regular meeting. The resolution of such a dispute by the Administrator shall be final and binding on the Company and on Participant. 

9. Governing Law; Severability. This Agreement shall be governed by and construed in accordance with the laws of the State of
California, excluding that body of law pertaining to conflicts of law. Should any provision of this Agreement be determined by a court of law to be illegal or unenforceable, the other provisions shall nevertheless remain effective and shall remain
enforceable. 

  
 B-5 

 10. Notices. Any notice required or permitted hereunder shall be given in accordance with
the provisions set forth in Section 5.6 of the Option Agreement. 
 11. Further Instruments. The parties agree to execute such
further instruments and to take such further action as may be reasonably necessary to carry out the purposes and intent of this Agreement. 

12. Entire Agreement. The Plan and Option Agreement are incorporated herein by reference. This Agreement, the Plan and the Option
Agreement constitute the entire agreement of the parties and supersede in their entirety all prior undertakings and agreements of the Company and Participant with respect to the subject matter hereof. 

 

									
	 ACCEPTED BY:
 ULTRAGENYX
PHARMACEUTICAL, INC.
	 		 	 SUBMITTED BY

PARTICIPANT:

					
	By:	 	 	 		 	By:	 	 

									
					
	Print Name:	 		 		 	Print Name:	 	 

									
					
	 Title:
	 		 		 		 	
					
		 		 		 	 Address:
	 	 
					
		 		 		 		 	 

  
 B-6 

 CONSENT OF SPOUSE 

CONSENT OF SPOUSE 
 I,
                    , spouse of
                    , have read and approve the Option Agreement and this Exercise Notice between my spouse and Ultragenyx Pharmaceutical,
Inc. In consideration of granting of the right to my spouse to purchase shares of Ultragenyx Pharmaceutical, Inc. set forth in the Option Agreement and this Exercise Notice, I hereby appoint my spouse as my attorney-in-fact in respect to the
exercise of any rights under the Option Agreement and this Exercise Notice and agree to be bound by the provisions of the Plan, the Option Agreement and this Exercise Notice insofar as I may have any rights in said agreements or any shares issued
pursuant thereto under the community property laws or similar laws relating to marital property in effect in the state of our residence as of the date of the signing of the foregoing Exercise Notice. 

 

							
	Dated:	 	                        ,
            	 		 	 
		 		 		 	Signature of Spouse

  
 B-7 

 ULTRAGENYX PHARMACEUTICAL, INC. 

2011 EQUITY INCENTIVE PLAN STOCK OPTION GRANT NOTICE AND 

STOCK OPTION AGREEMENT 

Ultragenyx Pharmaceutical, Inc. (the “Company”), pursuant to its 2011 Equity Incentive Plan (the
“Plan”), hereby grants to the holder listed below (“Participant”), an option to purchase the number of shares of the Company’s Stock set forth below (the “Option”). This
Option is subject to all of the terms and conditions as set forth herein and in the Stock Option Agreement attached hereto as Exhibit A (the “Stock Option Agreement”) and the Plan, each of which are incorporated herein
by reference. Unless otherwise defined herein, the terms defined in the Plan shall have the same defined meanings in this Grant Notice and the Stock Option Agreement. 
  

					
	 Participant:
	 	 
		
	 Grant Date:
	 	 
		
	 Vesting Commencement Date:
	 	 
			
	 Exercise Price per Share:
	 	 $  
	  	 
			
	 Total Exercise Price:
	 	 $  
	  	 
		
	 Total Number of Shares Subject to the Option:
	 	 
		
	 Expiration Date:
	 	 

 Type of
Option:   ̈      Incentive Stock Option                  ̈  Non-Qualified Stock Option 
 Exercise
Schedule:  x Early Exercise Permitted 
  

			
	Vesting Schedule:	  	This Option is exercisable immediately, in whole or in part, at such times as are established by the Administrator, conditioned upon Participant entering into a Restricted Stock Purchase Agreement with respect to any unvested shares
of Stock. The shares subject to this Option shall vest and/or be released from the Company’s Repurchase Option, as set forth in the Restricted Stock Purchase Agreement attached hereto as Exhibit C (the “Restricted Stock
Purchase Agreement”), according to the following schedule:
		
		  	[To be specified in individual agreements.]

 By his or her signature and the Company’s signature below, Participant agrees to be bound by the terms
and conditions of the Plan, the Stock Option Agreement and this Grant Notice. Participant has reviewed the Stock Option Agreement, the Plan and this Grant Notice in their entirety, has had an opportunity to obtain the advice of counsel prior to
executing this Grant Notice and fully understands all provisions of this Grant Notice, the Stock Option Agreement and the Plan. Participant hereby agrees to accept as binding, conclusive and final all decisions or interpretations of the
Administrator of the Plan upon any questions arising under the Plan or the Option. 
  

									
	ULTRAGENYX PHARMACEUTICAL, INC.	 		 	PARTICIPANT:
					
	By:	 	 	 		 	By:	 	 

									
					
	Print Name:	 		 		 	Print Name:	 	 

									
					
	 Title:
	 		 		 		 	
					
	 Address:
	 		 		 	 Address:
	 	 
					
		 		 		 		 	 
					
		 		 		 		 	 

 EXHIBIT A 

TO STOCK OPTION GRANT NOTICE 

STOCK OPTION AGREEMENT 

Pursuant to the Stock Option Grant Notice (“Grant Notice”) to which this Stock Option Agreement (this
“Agreement”) is attached, Ultragenyx Pharmaceutical, Inc. (the “Company”) has granted to Participant an option under the Company’s 2011 Equity Incentive Plan (the “Plan”)
to purchase the number of shares of Stock indicated in the Grant Notice. 
 ARTICLE I 

GENERAL 
 1.1 Defined
Terms. Capitalized terms not specifically defined herein shall have the meanings specified in the Plan and the Grant Notice. 
 1.2
Incorporation of Terms of Plan. The Option is subject to the terms and conditions of the Plan which are incorporated herein by reference. 

ARTICLE II 
 GRANT OF
OPTION 
 2.1 Grant of Option. In consideration of Participant’s past and/or continued employment with or service to the
Company or a Parent or Subsidiary and for other good and valuable consideration, effective as of the Grant Date set forth in the Grant Notice (the “Grant Date”), the Company irrevocably grants to Participant the Option to purchase
any part or all of an aggregate of the number of shares of Stock set forth in the Grant Notice, upon the terms and conditions set forth in the Plan and this Agreement. Unless designated as a Non-Qualified Stock Option in the Grant Notice, the Option
shall be an Incentive Stock Option to the maximum extent permitted by law. 
 2.2 Exercise Price. The exercise price of the
shares of Stock subject to the Option shall be as set forth in the Grant Notice, without commission or other charge; provided, however, that if this Option is designated as an Incentive Stock Option, the price per share of the shares subject
to the Option shall not be less than the greater of (i) 100% of the Fair Market Value of a share of Stock on the Grant Date, or (ii) 110% of the Fair Market Value of a share of Stock on the Grant Date in the case of a Participant then
owning (within the meaning of Section 424(d) of the Code) more than 10% of the total combined voting power of all classes of stock of the Company or any “subsidiary corporation” of the Company or any “parent corporation” of
the Company (each within the meaning of Section 424 of the Code). 

  
 A-1 

 ARTICLE III 

PERIOD OF EXERCISABILITY 

3.1 Commencement of Exercisability. 

(a) Subject to Sections 3.3 and 5.8, the Option shall become vested and exercisable in such amounts and at such times as are
set forth in the Grant Notice. Alternatively, at the election of the Participant, this Option may be exercised in whole or in part at such times as are established by the Administrator as to shares of Stock which have not yet vested. Vested shares
shall not be subject to the Company’s Repurchase Option (as set forth in the Restricted Stock Purchase Agreement). As a condition to exercising this Option for unvested shares of Stock, the Participant shall execute the Restricted Stock
Purchase Agreement. 
 (b) No portion of the Option which has not become vested and exercisable at the date of
Participant’s Termination of Service shall thereafter become vested and exercisable, except as may be otherwise provided by the Administrator or as set forth in a written agreement between the Company and Participant. 

3.2 Duration of Exercisability. The installments provided for in the vesting schedule set forth in the Grant Notice are cumulative.
Each such installment which becomes vested and exercisable pursuant to the vesting schedule set forth in the Grant Notice shall remain vested and exercisable until it becomes unexercisable under Section 3.3. 

3.3 Expiration of Option. The Option may not be exercised to any extent by anyone after the first to occur of the following events:

 (a) The expiration of ten years from the Grant Date; 

(b) If this Option is designated as an Incentive Stock Option and Participant owned (within the meaning of Section 424(d)
of the Code), at the time the Option was granted, more than 10% of the total combined voting power of all classes of stock of the Company or any “subsidiary corporation” of the Company or “parent corporation” of the Company (each
within the meaning of Section 424 of the Code), the expiration of five years from the date the Option was granted; or 

(c) The expiration of three months following the date of Participant’s Termination of Service, unless such termination
occurs by reason of Participant’s death, Disability or Misconduct; 
 (d) The expiration of one year following the date
of Participant’s Termination of Service by reason of Participant’s death or Disability; or 
 (e) The date of
Participant’s Termination of Service as a result of Participant’s Misconduct. 
 Participant acknowledges that an Incentive Stock
Option exercised more than three months after Participant’s termination of status as an Employee, other than by reason of death or Disability, will be taxed as a Non-Qualified Stock Option. 

  
 A-2 

 3.4 Special Tax Consequences. Participant acknowledges that, to the extent that the
aggregate Fair Market Value (determined as of the time the Option is granted) of all shares of Stock with respect to which Incentive Stock Options, including the Option, are first exercisable for the first time by Participant in any calendar year
exceeds $100,000 (or such other limitation as imposed by Section 422(d) of the Code), the Option and such other options shall be treated as not qualifying under Section 422 of the Code but rather shall be considered Non-Qualified Stock
Options. Participant further acknowledges that the rule set forth in the preceding sentence shall be applied by taking Options and other “incentive stock options” into account in the order in which they were granted. 

ARTICLE IV 
 EXERCISE OF
OPTION 
 4.1 Person Eligible to Exercise. Except as provided in Sections 5.2(b) and 5.2(c), during the lifetime of Participant,
only Participant may exercise the Option or any portion thereof. After the death of Participant, any exercisable portion of the Option may, prior to the time when the Option becomes unexercisable under Section 3.3, be exercised by
Participant’s personal representative or by any person empowered to do so under the deceased Participant’s will or under the then applicable laws of descent and distribution. 

4.2 Partial Exercise. Any exercisable portion of the Option or the entire Option, if then wholly exercisable, may be exercised in whole
or in part at any time prior to the time when the Option or portion thereof becomes unexercisable under Section 3.3. 
 4.3 Manner
of Exercise. The Option, or any exercisable portion thereof, may be exercised solely by delivery to the Secretary of the Company or the Secretary’s office of all of the following prior to the time when the Option or such portion thereof
becomes unexercisable under Section 3.3: 
 (a) An Exercise Notice in writing signed by Participant or any other person
then entitled to exercise the Option or portion thereof, stating that the Option or portion thereof is thereby exercised, such notice complying with all applicable rules established by the Administrator. Such notice shall be substantially in the
form attached as Exhibit B to the Grant Notice (or such other form as is prescribed by the Administrator); and 
 (b)
(b) A Restricted Stock Purchase Agreement, if applicable, substantially in the form attached as Exhibit C to the Grant Notice; 

(c) Subject to Section 5.1(c) of the Plan: 

(i) Full payment (in cash or by check) for the shares with respect to which the Option or portion thereof is exercised; or 

(ii) With the consent of the Administrator, by delivery of a full recourse promissory note on such terms and conditions as may
be approved by the Administrator; or 

  
 A-3 

 (iii) With the consent of the Administrator, by delivery of shares of Stock then
issuable upon exercise of the Option having a Fair Market Value on the date of delivery equal to the aggregate exercise price of the Option or exercised portion thereof; or 

(iv) On and after the Public Trading Date, such payment may be made, in whole or in part, through the delivery of shares of
Stock which have been owned by Participant for at least six months, duly endorsed for transfer to the Company with a Fair Market Value on the date of delivery equal to the aggregate exercise price of the Option or exercised portion thereof; or 

(v) On and after the Public Trading Date, through the delivery of a notice that Participant has placed a market sell order with
a broker with respect to shares of Stock then issuable upon exercise of the Option, and that the broker has been directed to pay a sufficient portion of the net proceeds of the sale to the Company in satisfaction of the Option exercise price;
provided, that payment of such proceeds is made to the Company upon settlement of such sale; or 
 (vi) Subject to any
applicable laws, any combination of the consideration provided in the foregoing paragraphs (i), (ii) and (iii); and 

(d) A bona fide written representation and agreement, in such form as is prescribed by the Administrator, signed by Participant
or the other person then entitled to exercise such Option or portion thereof, stating that the shares of Stock are being acquired for Participant’s own account, for investment and without any present intention of distributing or reselling said
shares or any of them except as may be permitted under the Securities Act and then applicable rules and regulations thereunder, and that Participant or other person then entitled to exercise such Option or portion thereof will indemnify the Company
against and hold it free and harmless from any loss, damage, expense or liability resulting to the Company if any sale or distribution of the shares by such person is contrary to the representation and agreement referred to above. The Administrator
may, in its absolute discretion, take whatever additional actions it deems appropriate to ensure the observance and performance of such representation and agreement and to effect compliance with the Securities Act and any other federal or state
securities laws or regulations. Without limiting the generality of the foregoing, the Administrator may require an opinion of counsel acceptable to it to the effect that any subsequent transfer of shares acquired on an Option exercise does not
violate the Securities Act, and may issue stop-transfer orders covering such shares. Share certificates evidencing Stock issued on exercise of the Option shall bear an appropriate legend referring to the provisions of this subsection (d) and
the agreements herein. The written representation and agreement referred to in the first sentence of this subsection (d) shall, however, not be required if the shares to be issued pursuant to such exercise have been registered under the
Securities Act, and such registration is then effective in respect of such shares; and 
 (e) The receipt by the Company of
full payment for such shares, including payment of any applicable withholding tax, which may be in the form of consideration used by Participant to pay for such shares under Section 4.3(b), subject to Section 15.3 of the Plan; and 

  
 A-4 

 (f) In the event the Option or portion thereof shall be exercised pursuant to
Section 4.1 by any person or persons other than Participant, appropriate proof of the right of such person or persons to exercise the Option. 

4.4 Conditions to Issuance of Stock Certificates. The shares of Stock deliverable upon the exercise of the Option, or any portion
thereof, may be either previously authorized but unissued shares or issued shares which have then been reacquired by the Company. Such shares shall be fully paid and nonassessable. The Company shall not be required to issue or deliver any shares of
Stock purchased upon the exercise of the Option or portion thereof prior to fulfillment of all of the following conditions: 

(a) The admission of such shares to listing on all stock exchanges on which such Stock is then listed; and 

(b) The completion of any registration or other qualification of such shares under any state or federal law or under rulings or
regulations of the Securities and Exchange Commission or of any other governmental regulatory body, which the Administrator shall, in its absolute discretion, deem necessary or advisable; and 

(c) The obtaining of any approval or other clearance from any state or federal governmental agency which the Administrator
shall, in its absolute discretion, determine to be necessary or advisable; and 
 (d) The lapse of such reasonable period of
time following the exercise of the Option as the Administrator may from time to time establish for reasons of administrative convenience; and 

(e) The receipt by the Company of full payment for such shares, including payment of any applicable withholding tax, which may
be in the form of consideration used by Participant to pay for such shares under Section 4.3(b), subject to Section 15.3 of the Plan. 

4.5 Rights as Stockholder. The holder of the Option shall not be, nor have any of the rights or privileges of, a stockholder of the
Company in respect of any shares purchasable upon the exercise of any part of the Option unless and until such shares shall have been issued by the Company to such holder. 

ARTICLE V 
 OTHER
PROVISIONS 
 5.1 Administration. The Administrator shall have the power to interpret the Plan and this Agreement and to adopt
such rules for the administration, interpretation and application of the Plan as are consistent therewith and to interpret, amend or revoke any such rules. All actions taken and all interpretations and determinations made by the Administrator in
good faith shall be final and binding upon Participant, the Company and all other interested persons. No member of the Administrator shall be personally liable for any action, determination or interpretation made in good faith with respect to the
Plan, this Agreement or the Option. In its absolute discretion, the Board may at any time and from time to time exercise any and all rights and duties of the Administrator under the Plan and this Agreement. 

  
 A-5 

 5.2 Option Not Transferable. 

(a) Subject to Section 5.2(b), the Option may not be sold, pledged, assigned or transferred in any manner other than by
will or the laws of descent and distribution, unless and until the shares underlying the Option have been issued, and all restrictions applicable to such shares have lapsed. Neither the Option nor any interest or right therein shall be liable for
the debts, contracts or engagements of Participant or his or her successors in interest or shall be subject to disposition by transfer, alienation, anticipation, pledge, encumbrance, assignment or any other means whether such disposition be
voluntary or involuntary or by operation of law by judgment, levy, attachment, garnishment or any other legal or equitable proceedings (including bankruptcy), and any attempted disposition thereof shall be null and void and of no effect, except to
the extent that such disposition is permitted by the preceding sentence. 
 (b) Notwithstanding any other provision in this
Agreement, with the consent of the Administrator and to the extent the Option is designated as a Non-Qualified Stock Option, the Option may be transferred to, exercised by and paid to one or more Permitted Transferees, subject to the terms and
conditions set forth in Section 10.3 of the Plan. 
 (c) Unless transferred to a Permitted Transferee in accordance with
Section 5.2(b), during the lifetime of Participant, only Participant may exercise the Option or any portion thereof. Subject to such conditions and procedures as the Administrator may require, a Permitted Transferee may exercise the Option or
any portion thereof during Participant’s lifetime. After the death of Participant, any exercisable portion of the Option may, prior to the time when the Option becomes unexercisable under Section 3.3, be exercised by Participant’s
personal representative or by any person empowered to do so under the deceased Participant’s will or under the then applicable laws of descent and distribution. 

5.3 Lock-Up Period. Participant hereby agrees that, if so requested by the Company or any representative of the
underwriters (the “Managing Underwriter”) in connection with any registration of the offering of any securities of the Company under the Securities Act, Participant shall not sell or otherwise transfer any shares of Stock or
other securities of the Company during such period as may be requested in writing by the Managing Underwriter and agreed to in writing by the Company (which period shall not be longer than one hundred eighty days) (the “Market Standoff
Period”) following the effective date of a registration statement of the Company filed under the Securities Act; provided, however, that such restriction shall apply only to the first registration
statement of the Company to become effective under the Securities Act that includes securities to be sold on behalf of the Company to the public in an underwritten public offering under the Securities Act. 

5.4 Restrictive Legends and Stop-Transfer Orders. 

(a) The share certificate or certificates evidencing the shares of Stock purchased hereunder shall be endorsed with any legends
that may be required by state or federal securities laws. 
 (b) Participant agrees that, in order to ensure compliance with
the restrictions referred to herein, the Company may issue appropriate “stop transfer” instructions to its transfer agent, if any, and that, if the Company transfers its own securities, it may make appropriate notations to the same effect
in its own records. 

  
 A-6 

 (c) The Company shall not be required: (i) to transfer on its books any
shares of Stock that have been sold or otherwise transferred in violation of any of the provisions of this Agreement, or (ii) to treat as owner of such shares of Stock or to accord the right to vote or pay dividends to any purchaser or other
transferee to whom such shares shall have been so transferred. 
 5.5 Shares to Be Reserved. The Company shall at all times during
the term of the Option reserve and keep available such number of shares of Stock as will be sufficient to satisfy the requirements of this Agreement. 

5.6 Notices. Any notice to be given under the terms of this Agreement to the Company shall be addressed to the Company in care of the
Secretary of the Company at the address given beneath the signature of the Company’s authorized officer on the Grant Notice, and any notice to be given to Participant shall be addressed to Participant at the address given beneath
Participant’s signature on the Grant Notice. By a notice given pursuant to this Section 5.6, either party may hereafter designate a different address for notices to be given to that party. Any notice which is required to be given to
Participant shall, if Participant is then deceased, be given to the person entitled to exercise his or her Option pursuant to Section 4.1 by written notice under this Section 5.6. Any notice shall be deemed duly given when sent via email
or when sent by certified mail (return receipt requested) and deposited (with postage prepaid) in a post office or branch post office regularly maintained by the United States Postal Service. 

5.7 Titles. Titles are provided herein for convenience only and are not to serve as a basis for interpretation or construction of this
Agreement. 
 5.8 Stockholder Approval. The Plan will be submitted for approval by the Company’s stockholders within twelve
months after the date the Plan was initially adopted by the Board. The Option may not be exercised to any extent by anyone prior to the time when the Plan is approved by the stockholders, and if such approval has not been obtained by the end of said
twelve month period, the Option shall thereupon be canceled and become null and void. 
 5.9 Governing Law; Severability. This
Agreement shall be administered, interpreted and enforced under the laws of the State of California, without regard to the conflicts of law principles thereof. Should any provision of this Agreement be determined by a court of law to be illegal or
unenforceable, the other provisions shall nevertheless remain effective and shall remain enforceable. 
 5.10 Conformity to Securities
Laws. Participant acknowledges that the Plan is intended to conform to the extent necessary with all provisions of the Securities Act and the Exchange Act and any and all regulations and rules promulgated by the Securities and Exchange
Commission thereunder, and state securities laws and regulations. Notwithstanding anything herein to the contrary, the Plan shall be administered, and the Option is granted and may be exercised, only in such a manner as to conform to such laws,
rules and regulations. To the extent permitted by applicable law, the Plan and this Agreement shall be deemed amended to the extent necessary to conform to such laws, rules and regulations. 

  
 A-7 

 5.11 Amendments. This Agreement may not be modified, amended or terminated except by an
instrument in writing, signed by Participant or such other person as may be permitted to exercise the Option pursuant to Section 4.1 and by a duly authorized representative of the Company. 

5.12 No Employment Rights. If Participant is an Employee, nothing in the Plan or this Agreement shall confer upon Participant any right
to continue in the employ of the Company or any Subsidiary or shall interfere with or restrict in any way the rights of the Company and its Subsidiaries, which are expressly reserved, to discharge Participant at any time for any reason whatsoever,
with or without cause, except to the extent expressly provided otherwise in a written agreement between the Company and Participant. 
 5.13
Successors and Assigns. The Company may assign any of its rights under this Agreement to single or multiple assignees, and this Agreement shall inure to the benefit of the successors and assigns of the Company. Subject to the restrictions on
transfer herein set forth, this Agreement shall be binding upon Participant and his or her heirs, executors, administrators, successors and assigns. 

5.14 Notification of Disposition. If this Option is designated as an Incentive Stock Option, Participant shall give prompt notice to
the Company of any disposition or other transfer of any shares of Stock acquired under this Agreement if such disposition or transfer is made (a) within two years from the Grant Date with respect to such shares or (b) within one year after
the transfer of such shares to him. Such notice shall specify the date of such disposition or other transfer and the amount realized, in cash, other property, assumption of indebtedness or other consideration, by Participant in such disposition or
other transfer. 
 5.15 Limitations Applicable to Section 16 Persons. Notwithstanding any other provision of the Plan or this
Agreement, if Participant is subject to Section 16 of the Exchange Act, the Plan, the Option, the Shares and this Agreement shall be subject to any additional limitations set forth in any applicable exemptive rule under Section 16 of the
Exchange Act (including any amendment to Rule 16b-3 of the Exchange Act) that are requirements for the application of such exemptive rule. To the extent permitted by applicable law, this Agreement shall be deemed amended to the extent necessary to
conform to such applicable exemptive rule. 
 5.16 Entire Agreement. The Plan and this Agreement (including all Exhibits hereto)
constitute the entire agreement of the parties and supersede in their entirety all prior undertakings and agreements of the Company and Participant with respect to the subject matter hereof. 

  
 A-8 

 EXHIBIT B 

TO STOCK OPTION GRANT NOTICE 

FORM OF EXERCISE NOTICE 

Effective as of today,         ,
                 the undersigned (“Participant”) hereby elects to exercise Participant’s option to purchase
                 shares of the Stock (the “Shares”) of Ultragenyx Pharmaceutical, Inc. (the “Company”) under and
pursuant to the Ultragenyx Pharmaceutical, Inc. 2011 Equity Incentive Plan (the “Plan”) and the Stock Option Grant Notice and Stock Option Agreement dated
            ,          (the “Option Agreement”). Capitalized terms used herein without definition shall have
the meanings given in the Option Agreement. 
  

							
	 Grant Date:
	 		 	 
			
	 Number of Shares as to which Option is Exercised:
	 		 	 
				
	 Exercise Price per Share:
	 		 	 $  
	  	 
				
	 Total Exercise Price:
	 		 	 $  
	  	 
			
	 Certificate to the issued in the name of:
	 		 	 
			
	 Cash Payment delivered herewith:
	 		 	$ __________________ (Representing the full Exercise Price for the Shares, as well as any applicable withholding tax)

 Type of
Option:   ̈      Incentive Stock Option                  ̈  Non-Qualified Stock Option 
 1. Representations of Participant. Participant
acknowledges that Participant has received, read and understood the Plan and the Option Agreement. Participant agrees to abide by and be bound by their terms and conditions. 

2. Rights as Stockholder. Until the Shares are issued (as evidenced by the appropriate entry on the books of the Company or of a duly
authorized transfer agent of the Company), no right to vote or receive dividends or any other rights as a stockholder shall exist with respect to Shares subject to the Option, notwithstanding the exercise of the Option. No adjustment will be made
for a dividend or other right for which the record date is prior to the date the Shares are issued, except as provided in Article 10 of the Plan. 

Participant shall enjoy rights as a stockholder until such time as Participant disposes of the Shares or the Company and/or its assignee(s)
exercises the Right of First Refusal hereunder. Upon such exercise, Participant shall have no further rights as a holder of the Shares so purchased except the right to receive payment for the Shares so purchased in accordance with the provisions of
this Agreement, and Participant shall forthwith cause the certificate(s), if any issued, evidencing the Shares so purchased to be surrendered to the Company for transfer or cancellation. 

  
 B-1 

 3. Participant’s Rights to Transfer Shares. 

(a) Before any Shares held by Participant or any permitted transferee (each, a “Holder”)
may be sold, pledged, assigned, hypothecated, transferred, or otherwise disposed of (each, a “Transfer”), the Company or its assignee(s) shall have a right of first refusal to purchase the Shares proposed to be Transferred on
the terms and conditions set forth in this Section (the “Right of First Refusal”). In the event that the Company’s Bylaws contain a right of first refusal with respect to the Shares, such right of first refusal shall
apply to the Shares to the extent such provisions are more restrictive than the Right of First Refusal set forth in this Section and the Right of First Refusal set forth in this Section shall not in any way restrict the operation of the
Company’s Bylaws. 
 (b) In the event any Holder desires to Transfer any Shares, the Holder
shall deliver to the Company a written notice (the “Notice”) stating: (i) the Holder’s bona fide intention to sell or otherwise Transfer such Shares; (ii) the name of each proposed purchaser or other transferee
(“Proposed Transferee”); (iii) the number of Shares to be Transferred to each Proposed Transferee; and (iv) the price for which the Holder proposes to Transfer the Shares (the “Offered
Price”), and the Holder shall offer such Shares at the Offered Price to the Company or its assignee(s). 

(c) Within twenty-five days after receipt of the Notice, the Company and/or its assignee(s) may elect in writing
to purchase all, but not less than all, of the Shares proposed to be Transferred to any one or more of the Proposed Transferees by delivery of a written exercise notice to the Holder (a “Company Notice”). The purchase price
will be determined in accordance with subsection (d) below. 
 (d) The purchase price
(“Purchase Price”) for the Shares repurchased under this Section shall be the Offered Price. 

(e) Payment of the Purchase Price shall be made, at the option of the Company or its assignee(s), in cash (by check), by
cancellation of all or a portion of any outstanding indebtedness of the Holder to the Company (or, in the case of repurchase by an assignee, to the assignee), or by any combination thereof within five days after delivery of the Company Notice or in
the manner and at the times mutually agreed to by the Company and the Holder. Should the Offered Price specified in the Notice be payable in property other than cash, the Company shall have the right to pay the purchase price in the form of cash
equal in amount to the value of such property. If the Holder and the Company cannot agree on such cash value within ten days after the Company’s receipt of the Notice, the valuation shall be made by the Board. The payment of the purchase price
shall then be held on the later of (i) five days following delivery of the Company Notice or (ii) five days after such valuation shall have been made. 

(f) If all or a portion of the Shares proposed in the Notice to be Transferred are not purchased by the Company and/or its
assignee(s) as provided in this Section, then the Holder may sell or otherwise Transfer such Shares to that Proposed Transferee at the Offered Price or at a higher price, provided that such sale or other Transfer is consummated within sixty days
after the date of the Notice and provided further that any such sale or other Transfer is 

  
 B-2 

 
effected in accordance with any applicable securities laws and the Proposed Transferee agrees in writing that the provisions of this Section shall continue to apply to the Shares in the hands of
such Proposed Transferee. If the Shares described in the Notice are not Transferred to the Proposed Transferee within such sixty-day period, a new Notice shall be given to the Company, and the Company and/or its assignees shall again be offered the
Right of First Refusal as provided herein before any Shares held by the Holder may be sold or otherwise Transferred. 

(g) Anything to the contrary contained in this Section notwithstanding, the Transfer of any or all of the Shares
during Participant’s lifetime or upon Participant’s death by will or intestacy to Participant’s Immediate Family or a trust for the benefit of Participant’s Immediate Family shall be exempt from the Right of First Refusal. As
used herein, “Immediate Family” shall mean spouse, lineal descendant or antecedent, father, mother, brother or sister or stepchild (whether or not adopted). In such case, the transferee or other recipient shall receive and
hold the Shares so Transferred subject to the provisions of this Section (including the Right of First Refusal) and the Restricted Stock Purchase Agreement, if applicable, and there shall be no further Transfer of such Shares except in accordance
with the terms of this Section. 
 (h) The Right of First Refusal shall terminate as to all Shares upon the
Public Trading Date. 
 (i) Any transfer or sale of the Shares is subject to restrictions on transfer imposed by any
applicable state and federal securities laws. Any Transfer or attempted Transfer of any of the Shares not in accordance with the terms of this Agreement shall be void and the Company may enforce the terms of this Agreement by stop transfer
instructions or similar actions by the Company and its agents or designees. 
 4. Tax Consultation. Participant understands that
Participant may suffer adverse tax consequences as a result of Participant’s purchase or disposition of the Shares. Participant represents that Participant has consulted with any tax consultants Participant deems advisable in connection with
the purchase or disposition of the Shares and that Participant is not relying on the Company for any tax advice. 
 5. Restrictive
Legends and Stop-Transfer Orders. 
 (a) Legends. Participant understands and agrees that the Company shall cause
any certificates issued evidencing the Shares shall have the legends set forth below or legends substantially equivalent thereto, together with any other legends that may be required by state or federal securities laws: 

THE SHARES REPRESENTED HEREBY HAVE NOT BEEN REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED (“ACT”), NOR HAVE THEY BEEN
REGISTERED OR QUALIFIED UNDER THE SECURITIES LAWS OF ANY STATE. NO TRANSFER OF SUCH SECURITIES WILL BE PERMITTED UNLESS A REGISTRATION STATEMENT UNDER THE ACT IS IN EFFECT AS TO SUCH TRANSFER, THE TRANSFER IS MADE IN ACCORDANCE WITH RULE 144 UNDER
THE ACT, OR IN THE 

  
 B-3 

 
OPINION OF COUNSEL (WHICH MAY BE COUNSEL FOR THE COMPANY) REGISTRATION UNDER THE ACT IS UNNECESSARY IN ORDER FOR SUCH TRANSFER TO COMPLY WITH THE ACT AND WITH APPLICABLE STATE SECURITIES LAWS.

 THE SHARES REPRESENTED BY THIS CERTIFICATE MAY BE TRANSFERRED ONLY IN ACCORDANCE WITH THE TERMS OF AN AGREEMENT BETWEEN THE COMPANY AND
THE STOCKHOLDER, A COPY OF WHICH IS ON FILE WITH THE SECRETARY OF THE COMPANY. SUCH TRANSFER RESTRICTIONS ARE BINDING ON TRANSFEREES OF THESE SHARES. 

(b) Participant agrees that, in order to ensure compliance with the restrictions referred to herein, the Company may issue
appropriate “stop transfer” instructions to its transfer agent, if any, and that, if the Company transfers its own securities, it may make appropriate notations to the same effect in its own records. 

(c) The Company shall not be required (i) to transfer on its books any Shares that have been sold or otherwise transferred
in violation of any of the provisions of this Agreement or (ii) to treat as owner of such Shares or to accord the right to vote or pay dividends to any purchaser or other transferee to whom such Shares shall have been so transferred. 

6. Participant Representations. Participant hereby makes the following certifications and representations with respect to the Shares
listed above: 
 (a) Participant is aware of the Company’s business affairs and financial condition and has acquired
sufficient information about the Company to reach an informed and knowledgeable decision to acquire the Shares. Participant is acquiring these Shares for investment for Participant’s own account only and not with a view to, or for resale in
connection with, any “distribution” thereof within the meaning of the Securities Act. 
 (b) Participant
acknowledges and understands that the Shares constitute “restricted securities” under the Securities Act and have not been registered under the Securities Act in reliance upon a specific exemption therefrom, which exemption depends upon,
among other things, the bona fide nature of Participant’s investment intent as expressed herein. Participant understands that the Shares must be held indefinitely unless they are subsequently registered under the Securities Act or an exemption
from such registration is available. Participant further acknowledges and understands that the Company is under no obligation to register the Shares. Participant understands that the certificate evidencing the Shares will be imprinted with a legend
which prohibits the transfer of the Shares unless they are registered or such registration is not required in the opinion of counsel satisfactory to the Company and any other legend required under applicable state securities laws. 

(c) Participant is familiar with the provisions of Rule 701 and Rule 144, each promulgated under the Securities Act, which, in
substance, permit limited public resale of “restricted securities” acquired, directly or indirectly from the issuer thereof, in a non-public 

  
 B-4 

 
offering subject to the satisfaction of certain conditions. Rule 701 provides that if the issuer qualifies under Rule 701 at the time of the grant of the Option to Participant, the exercise will
be exempt from registration under the Securities Act. In the event the Company becomes subject to the reporting requirements of Section 13 or 15(d) of the Exchange Act, ninety days thereafter (or such longer period as any market stand-off
agreement may require) the securities exempt under Rule 701 may be resold, subject to the satisfaction of certain of the conditions specified by Rule 144, including: (i) the resale being made through a broker in an unsolicited
“broker’s transaction” or in transactions directly with a market maker (as said term is defined under the Exchange Act); and, in the case of an affiliate, (ii) the availability of certain public information about the Company,
(iii) the amount of securities being sold during any three month period not exceeding the limitations specified in Rule 144(e), and (iv) the timely filing of a Form 144, if applicable. 

(d) In the event that the Company does not qualify under Rule 701 at the time of grant of the Option, then the securities may
be resold in certain limited circumstances subject to the provisions of Rule 144, which requires the resale to occur not less than one year after the later of the date the securities were sold by the Company or the date the securities were sold by
an affiliate of the Company, within the meaning of Rule 144; and, in the case of acquisition of the securities by an affiliate, or by a non-affiliate who subsequently holds the securities less than two years, the satisfaction of the conditions set
forth in sections (i), (ii), (iii) and (iv) of paragraph (c) above. 
 (e) Participant further understands
that in the event all of the applicable requirements of Rule 701 or 144 are not satisfied, registration under the Securities Act, compliance with Regulation A, or some other registration exemption will be required; and that, notwithstanding the fact
that Rules 144 and 701 are not exclusive, the Staff of the Securities and Exchange Commission has expressed its opinion that persons proposing to sell private placement securities other than in a registered offering and otherwise than pursuant to
Rules 144 or 701 will have a substantial burden of proof in establishing that an exemption from registration is available for such offers or sales, and that such persons and their respective brokers who participate in such transactions do so at
their own risk. Participant understands that no assurances can be given that any such other registration exemption will be available in such event. 

7. Successors and Assigns. The Company may assign any of its rights under this Agreement to single or multiple assignees, and this
Agreement shall inure to the benefit of the successors and assigns of the Company. Subject to the restrictions on transfer herein set forth, this Agreement shall be binding upon Participant and his or her heirs, executors, administrators, successors
and assigns. 
 8. Interpretation. Any dispute regarding the interpretation of this Agreement shall be submitted by Participant or by
the Company forthwith to the Administrator, which shall review such dispute at its next regular meeting. The resolution of such a dispute by the Administrator shall be final and binding on the Company and on Participant. 

9. Governing Law; Severability. This Agreement shall be governed by and construed in accordance with the laws of the State of
California, excluding that body of law pertaining to conflicts of law. Should any provision of this Agreement be determined by a court of law to be illegal or unenforceable, the other provisions shall nevertheless remain effective and shall remain
enforceable. 

  
 B-5 

 10. Notices. Any notice required or permitted hereunder shall be given in accordance with
the provisions set forth in Section 5.6 of the Option Agreement. 
 11. Further Instruments. The parties agree to execute such
further instruments and to take such further action as may be reasonably necessary to carry out the purposes and intent of this Agreement. 

12. Entire Agreement. The Plan and Option Agreement are incorporated herein by reference. This Agreement, the Plan and the Option
Agreement constitute the entire agreement of the parties and supersede in their entirety all prior undertakings and agreements of the Company and Participant with respect to the subject matter hereof. 

 

									
	 ACCEPTED BY:
 ULTRAGENYX
PHARMACEUTICAL, INC.
	 		 	 SUBMITTED BY

PARTICIPANT:

					
	By:	 	 	 		 	By:	 	 

									
					
	Print Name:	 		 		 	Print Name:	 	 

									
					
	 Title:
	 		 		 		 	
					
		 		 		 	 Address:
	 	 
					
		 		 		 		 	 

  
 B-6 

 CONSENT OF SPOUSE 

I,                     , spouse of
                    , have read and approve the Option Agreement and this Exercise Notice between my spouse and Ultragenyx Pharmaceutical,
Inc. In consideration of granting of the right to my spouse to purchase shares of Ultragenyx Pharmaceutical, Inc. set forth in the Option Agreement and this Exercise Notice, I hereby appoint my spouse as my attorney-in-fact in respect to the
exercise of any rights under the Option Agreement and this Exercise Notice and agree to be bound by the provisions of the Plan, the Option Agreement and this Exercise Notice insofar as I may have any rights in said agreements or any shares issued
pursuant thereto under the community property laws or similar laws relating to marital property in effect in the state of our residence as of the date of the signing of the foregoing Exercise Notice. 

 

							
	Dated:	 	                        ,
            	 		 	 
		 		 		 	Signature of Spouse

  
 B-7 

 EXHIBIT C 

TO STOCK OPTION GRANT NOTICE 

RESTRICTED STOCK PURCHASE AGREEMENT 

THIS RESTRICTED STOCK PURCHASE AGREEMENT is made between (the “Purchaser”) and Ultragenyx Pharmaceutical, Inc. (the
“Company”), as of             ,             . 

RECITALS 
 (1) Pursuant to
the exercise of the Option granted to Purchaser under the Company’s 2011 Equity Incentive Plan (the “Plan”) and pursuant to the Stock Option Agreement (the “Option Agreement”) dated
                ,             , by and between the Company and Purchaser with respect to such
grant, which Option Agreement is hereby incorporated by reference, Purchaser has elected to purchase of those shares which have not become vested under the vesting schedule set forth in the Option Agreement (“Unvested
Shares”). The Unvested Shares and the shares subject to the Option Agreement which have become vested are sometimes collectively referred to herein as the “Shares”. 

(2) As required by the Option Agreement, as a condition to Purchaser’s election to exercise the option, Purchaser must execute this
Restricted Stock Purchase Agreement, which sets forth the rights and obligations of the parties with respect to Shares acquired upon exercise of the Option. 

1. Repurchase Option. 

(a) In the event of Purchaser’s Termination of Service (as defined in the Option Agreement), for any reason,
including for cause, death, Disability or Misconduct, the Company shall have the right and option to purchase from Purchaser, or Purchaser’s personal representative, as the case may be, all of Purchaser’s Unvested Shares as of the date of
the Purchaser’s Termination of Service at the exercise price paid by Purchaser for such Shares in connection with the exercise of the Option (the “Repurchase Option”). 

(b) The Company may exercise its Repurchase Option by delivering, personally or by registered mail, to Purchaser (or his or her
transferee or legal representative, as the case may be), within ninety days of the date of the Purchaser’s Termination of Service, a notice in writing indicating the Company’s intention to exercise the Repurchase Option and setting forth a
date for closing not later than thirty days from the mailing of such notice. The closing shall take place at the Company’s office. At the closing, the holder of the certificates for the Unvested Shares being transferred shall deliver the stock
certificate or certificates evidencing the Unvested Shares, and the Company shall deliver the purchase price therefor. 
 (c)
At its option, the Company may elect to make payment for the Unvested Shares to a bank selected by the Company. The Company shall avail itself of this option by a notice in writing to Purchaser stating the name and address of the bank, date of
closing, and waiving the closing at the Company’s office. 

  
 C-1 

 (d) If the Company does not elect to exercise the Repurchase Option conferred
above by giving the requisite notice within ninety days following the date of Purchaser’s Termination of Service, the Repurchase Option shall terminate. 

(e) 100% of the Unvested Shares shall initially be subject to the Repurchase Option. The Unvested Shares shall be released from
the Repurchase Option in accordance with the Vesting Schedule set forth in the Option Agreement until all Shares are released from the Repurchase Option. Fractional Shares shall be rounded down to the nearest whole share. 

2. Transferability of the Shares; Escrow. 

(a) Purchaser hereby authorizes and directs the secretary of the Company, or such other person designated by the Company from
time to time, to transfer the Unvested Shares as to which the Repurchase Option has been exercised from Purchaser to the Company. 

(b) To insure the availability for delivery of Purchaser’s Unvested Shares upon repurchase by the Company pursuant
to the Repurchase Option under Section 1, Purchaser hereby appoints the assistant secretary, or any other person designated by the Company from time to time as escrow agent, as its attorney-in-fact to sell, assign and transfer unto the Company,
such Unvested Shares, if any, repurchased by the Company pursuant to the Repurchase Option. If certificates for the Shares are issued, then Purchaser shall, upon execution of this Agreement, deliver and deposit with the assistant secretary of the
Company, or such other person designated by the Company from time to time, any share certificate(s) issued representing the Unvested Shares, together with the stock assignment duly endorsed in blank, attached hereto as Exhibit C-1. The Unvested Shares and stock assignment shall be held by the assistant secretary in escrow, pursuant to the Joint Escrow Instructions of the Company and Purchaser attached as Exhibit C-2 hereto,
until the Company exercises its Repurchase Option as provided in Section 1, until such Unvested Shares are vested, or until such time as this Agreement no longer is in effect. As a further condition to the Company’s obligations under this
Agreement, the spouse of Purchaser, if any, shall execute and deliver to the Company the Consent of Spouse set forth on the signature page hereto. Upon vesting of the Unvested Shares, the escrow agent shall promptly deliver to Purchaser the
certificate or certificates representing such Shares in the escrow agent’s possession belonging to Purchaser, and the escrow agent shall be discharged of all further obligations hereunder; provided, however, that the escrow agent shall
nevertheless retain such certificate or certificates as escrow agent if so required pursuant to other restrictions imposed pursuant to this Agreement. If the Shares are held in book entry form, then such entry will reflect that the Shares are
subject to the restrictions of this Agreement. 
 (c) The Company, or its designee, shall not be liable for any act it
may do or omit to do with respect to holding the Shares in escrow and while acting in good faith and in the exercise of its judgment. 

(d) Transfer or sale of the Shares is subject to restrictions on transfer imposed by any applicable state and federal
securities laws. Any transferee shall hold such Shares subject to all the provisions hereof and the Exercise Notice executed by Purchaser with respect to any Unvested Shares purchased by Purchaser and shall acknowledge the same by signing a copy of
this Agreement. Any transfer or attempted transfer of any of the Shares not in accordance with the terms of this Agreement shall be void and the Company may enforce the terms of this Agreement by stop transfer instructions or similar actions by the
Company and its agents or designees. 

  
 C-2 

 3. Ownership, Voting Rights, Duties. This Agreement shall not affect in any way the
ownership, voting rights or other rights or duties of Purchaser, except as specifically provided herein. 
 4. Legends. Any share
certificate evidencing the Shares issued hereunder shall be endorsed with the following legend (in addition to any legend required under applicable securities laws): 

THE SHARES REPRESENTED BY THIS CERTIFICATE MAY BE TRANSFERRED ONLY IN ACCORDANCE WITH THE TERMS OF AN AGREEMENT BETWEEN THE COMPANY AND THE
STOCKHOLDER, A COPY OF WHICH IS ON FILE WITH THE SECRETARY OF THE COMPANY. 
 5. Adjustment for Stock Split. In the event of any
stock dividend, stock split, reverse stock split, recapitalization, combination, reclassification, or similar change in the capital structure of the Company, the Administrator shall make appropriate and equitable adjustments in the Unreleased Shares
subject to the Repurchase Option and the number of Shares, consistent with any adjustment under Section 11.1 of the Plan. The provisions of this Agreement shall apply, to the full extent set forth herein with respect to the Shares, to any and
all shares of capital stock or other securities or other property or cash which may be issued in respect of, in exchange for, or in substitution of the Shares, and shall be appropriately adjusted for any stock dividends, splits, reverse splits,
combinations, recapitalizations and the like occurring after the date hereof. 
 6. Notices. Notices required hereunder shall be
given in person or by registered mail to the address of Purchaser shown on the records of the Company, and to the Company at its principal executive office. 

7. Survival of Terms. This Agreement shall apply to and bind Purchaser and the Company and their respective permitted assignees and
transferees, heirs, legatees, executors, administrators and legal successors. 
 8. Section 83(b) Elections. 

(a) Election for Unvested Shares Purchased Pursuant to a Non-Qualified Stock Option. Purchaser hereby acknowledges that
he or she has been informed that, with respect to the exercise of a Non-Qualified Stock Option for Unvested Shares, that unless an election is filed by Purchaser with the Internal Revenue Service and, if necessary, the proper state taxing
authorities, within thirty days of the purchase of the Shares, electing pursuant to Section 83(b) of the Code (and similar state tax provisions if applicable) to be taxed currently on any difference between the purchase price of the
Shares and their Fair Market Value on the date of purchase, there will be a recognition of taxable income to the Purchaser, measured by the excess, if any, of the fair market value of the Shares, at the time the Company’s Repurchase Option
lapses over the purchase price for the Shares. Purchaser represents that Purchaser has consulted any tax consultant(s) Purchaser deems advisable in connection with the purchase of the Shares or the filing of the election under Section 83(b) and
similar tax provisions. 

  
 C-3 

 (b) Election for Unvested Shares Purchased Pursuant to an Incentive Stock
Option. Purchaser hereby acknowledges that he or she has been informed that, with respect to the exercise of an Incentive Stock Option for Unvested Shares, that unless an election is filed by Purchaser with the Internal Revenue Service and, if
necessary, the proper state taxing authorities, within thirty days of the purchase of the Shares, electing pursuant to Section 83(b) of the Code (and similar state tax provisions if applicable) to be taxed currently on any difference
between the purchase price of the Shares and their Fair Market Value on the date of purchase, there will be a recognition of income to the Purchaser, for alternative minimum tax purposes measured by the excess, if any, of the fair market value of
the Shares at the time the Company’s Repurchase Option lapses over the purchase price for the Shares. Purchaser further acknowledges that if an election is filed under Section 83(b) of the Code for the Unvested Shares and such shares are
sold or transferred prior to the date two years following the Grant Date and one year following the purchase date of such shares, there will be a recognition of income to the Purchaser, for ordinary income, measured by the excess, if any, of the
fair market value of the Shares at the time the Company’s Repurchase Option lapses over the purchase price for the Shares. Purchaser represents that Purchaser has consulted any tax consultant(s) Purchaser deems advisable in connection with the
purchase of the Shares or the filing of the election under Section 83(b) and similar tax provisions. 
 PURCHASER ACKNOWLEDGES THAT
IT IS PURCHASER’S SOLE RESPONSIBILITY AND NOT THE COMPANY’S TO FILE TIMELY THE ELECTION UNDER SECTION 83(b), EVEN IF PURCHASER REQUESTS THE COMPANY OR ITS REPRESENTATIVE TO MAKE THIS FILING ON PURCHASER’S BEHALF. 

9. Representations. Purchaser has reviewed with his or her own tax advisors the federal, state, local and foreign tax consequences of
this investment and the transactions contemplated by this Agreement. Purchaser is relying solely on such advisors and not on any statements or representations of the Company or any of its agents. Purchaser understands that Purchaser (and not the
Company) shall be responsible for his or her own tax liability that may arise as a result of this investment or the transactions contemplated by this Agreement. 

10. Governing Law; Severability. This Agreement shall be governed by and construed in accordance with the laws of the State of
California excluding that body of law pertaining to conflicts of law. Should any provision of this Agreement be determined by a court of law to be illegal or unenforceable, the other provisions shall nevertheless remain effective and shall remain
enforceable. 
 Purchaser represents that he or she has read this Agreement and is familiar with its terms and provisions. Purchaser hereby
agrees to accept as binding, conclusive and final all decisions or interpretations of the Administrator upon any questions arising under this Agreement. 

(Signature Page Follows) 

  
 C-4 

 IN WITNESS WHEREOF, this Agreement is deemed made as of the date first set forth above. 

 

			
	ULTRAGENYX PHARMACEUTICAL, INC.
		
	By:	 	 
		
	Title:	 	 
	
	PURCHASER
		
	By:	 	 
		
	Title:	 	 
	
	Address:
		
		 	 
		
		 	 

  
 C-5 

 CONSENT OF SPOUSE 

I,                     , spouse of
                    , have read and approve this Agreement between my spouse and Ultragenyx Pharmaceutical, Inc. In consideration of granting
of the right to my spouse to purchase shares of Ultragenyx Pharmaceutical, Inc. set forth in the Agreement, I hereby appoint my spouse as my attorney-in-fact in respect to the exercise of any rights under the Agreement and agree to be bound by the
provisions of the Plan, the Option Agreement and this Agreement insofar as I may have any rights in said agreements or any shares issued pursuant thereto under the community property laws or similar laws relating to marital property in effect in the
state of our residence as of the date of the signing of the foregoing Agreement. 
  

			
	Signature of Spouse

  
 C-6 

 EXHIBIT C-1 

TO RESTRICTED STOCK PURCHASE AGREEMENT 

ASSIGNMENT SEPARATE FROM CERTIFICATE 

FOR VALUE RECEIVED, the undersigned,             , hereby sells, assigns
and transfers unto Ultragenyx Pharmaceutical, Inc., a California corporation (the “Company”),                  shares of the common stock
of the Company standing in its name of the books of said corporation represented by Certificate No.              herewith and do hereby irrevocably constitute and appoint to
                 transfer the said stock on the books of the within named corporation with full power of substitution in the premises. 

This Stock Assignment may be used only in accordance with the Restricted Stock Award Agreement between the Company and the undersigned dated ,
                .              

Dated:                 ,
             
  

			
	 	 
	Print: 	 	 

 
			
		
	 Name:
	 	

 INSTRUCTIONS: Please do not fill in the blanks other than the signature line. The purpose of this
assignment is to enable the Company to exercise its “Repurchase Option,” as set forth in the Restricted Stock Award Agreement, without requiring additional signatures on the part of Purchaser. 

  
 C-1-1 

 EXHIBIT C-2 

TO RESTRICTED STOCK PURCHASE AGREEMENT 

JOINT ESCROW INSTRUCTIONS 

                ,
             
 Secretary 

Ultragenyx Pharmaceutical, Inc. 
 77 Digital Drive, Suite 210 

Novato, CA 94949 
 As Escrow Agent
for both Ultragenyx Pharmaceutical, Inc. (the “Company”) and the undersigned purchaser of stock of the Company (the “Purchaser”), you are hereby authorized and directed to hold the documents delivered
to you pursuant to the terms of that certain Restricted Stock Purchase Agreement (“Agreement”) between the Company and the undersigned, in accordance with the following instructions: 

1. In the event the Company and/or any assignee of the Company (referred to collectively for convenience herein as the
“Company”) exercises the Company’s Repurchase Option set forth in the Agreement, the Company shall give to Purchaser and you a written notice specifying the number of shares of stock to be purchased, the purchase price,
and the time for a closing hereunder at the principal office of the Company. Purchaser and the Company hereby irrevocably authorize and direct you to close the transaction contemplated by such notice in accordance with the terms of said
notice. 
 2. At the closing, you are directed (a) to date the stock assignments necessary for the transfer in question,
(b) to fill in the number of shares being transferred, and (c) to deliver the same, together with the certificate evidencing the shares of stock to be transferred, to the Company or its assignee, against the simultaneous delivery to you of
the purchase price (by cash, a check, or a combination thereof) for the number of shares of stock being purchased pursuant to the exercise of the Company’s Repurchase Option. 

3. Purchaser irrevocably authorizes the Company to deposit with you any certificates evidencing shares of stock to be held by you hereunder
and any additions and substitutions to said shares as defined in the Agreement. Purchaser does hereby irrevocably constitute and appoint you as Purchaser’s attorney-in-fact and agent for the term of this escrow to execute, with respect to such
securities, all documents necessary or appropriate to make such securities negotiable and to complete any transaction herein contemplated, including but not limited to the filing with any applicable state blue sky authority of any required
applications for consent to, or notice of transfer of, the securities. Subject to the provisions of this paragraph 3, Purchaser shall exercise all rights and privileges of a stockholder of the Company while the stock is held by you. 

  
 C-2-1 

 4. Upon written request of Purchaser, but no more than once per calendar year, unless the
Company’s Repurchase Option has been exercised, you will deliver to Purchaser a certificate or certificates representing the number of shares of stock as are not then subject to the Company’s Repurchase Option. Within one hundred twenty
days after the date of the Purchaser’s Termination of Service, you will deliver to Purchaser a certificate or certificates representing the aggregate number of shares held or issued pursuant to the Agreement and not purchased by the Company or
its assignees pursuant to exercise of the Company’s Repurchase Option. 
 5. If at the time of termination of this escrow you should
have in your possession any documents, securities, or other property belonging to Purchaser, you shall deliver all of the same to Purchaser and shall be discharged of all further obligations hereunder. 

6. Your duties hereunder may be altered, amended, modified or revoked only by a writing signed by all of the parties hereto. 

7. You shall be obligated only for the performance of such duties as are specifically set forth herein and may rely and shall be protected in
relying or refraining from acting on any instrument reasonably believed by you to be genuine and to have been signed or presented by the proper party or parties. You shall not be personally liable for any act you may do or omit to do hereunder as
Escrow Agent or as attorney-in-fact for Purchaser while acting in good faith, and any act done or omitted by you pursuant to the advice of your own attorneys shall be conclusive evidence of such good faith. 

8. You are hereby expressly authorized to disregard any and all warnings given by any of the parties hereto or by any other person or
corporation, excepting only orders or process of courts of law and are hereby expressly authorized to comply with and obey orders, judgments or decrees of any court. In case you obey or comply with any such order, judgment or decree, you shall not
be liable to any of the parties hereto or to any other person, firm or corporation by reason of such compliance, notwithstanding any such order, judgment or decree being subsequently reversed, modified, annulled, set aside, vacated or found to have
been entered without jurisdiction. 
 9. You shall not be liable in any respect on account of the identity, authorities or rights of the
parties executing or delivering or purporting to execute or deliver the Agreement or any documents or papers deposited or called for hereunder. 

10. You shall not be liable for the expiration of any rights under any applicable state, federal or local statute of limitations or similar
statute or regulation with respect to these Joint Escrow Instructions or any documents deposited with you. 
 11. You shall be entitled to
employ such legal counsel and other experts as you may deem necessary properly to advise you in connection with your obligations hereunder, may rely upon the advice of such counsel, and may pay such counsel reasonable compensation therefore. 

12. Your responsibilities as Escrow Agent hereunder shall terminate if you shall cease to be an officer or agent of the Company or if you
shall resign by written notice to each party. In the event of any such termination, the Company shall appoint a successor Escrow Agent. 

  
 C-2-2 

 13. If you reasonably require other or further instruments in connection with these Joint Escrow
Instructions or obligations in respect hereto, the necessary parties hereto shall join in furnishing such instruments. 
 14. It is
understood and agreed that should any dispute arise with respect to the delivery and/or ownership or right of possession of the securities held by you hereunder, you are authorized and directed to retain in your possession without liability to
anyone all or any part of said securities until such disputes shall have been settled either by mutual written agreement of the parties concerned or by a final order, decree or judgment of a court of competent jurisdiction after the time for appeal
has expired and no appeal has been perfected, but you shall be under no duty whatsoever to institute or defend any such proceedings. 
 15.
Any notice required or permitted hereunder shall be given in writing and shall be deemed effectively given upon personal delivery or upon deposit in the United States Post Office, by registered or certified mail with postage and fees prepaid,
addressed to each of the other parties thereunto entitled at such addresses as a party may designate by written notice to each of the other parties hereto. 

16. By signing these Joint Escrow Instructions, you become a party hereto only for the purpose of said Joint Escrow Instructions; you do not
become a party to the Agreement. 
 17. This instrument shall be binding upon and inure to the benefit of the parties hereto, and their
respective successors and permitted assigns. 
 18. These Joint Escrow Instructions shall be governed by, and construed and enforced in
accordance with, the laws of the State of California, excluding that body of law pertaining to conflicts of law. 
 (Signature Page
Follows) 

  
 C-2-3 

 IN WITNESS WHEREOF, these Joint Escrow Instructions shall be effective as of the date first set
forth above. 
  

			
	 Very truly yours,
  

	ULTRAGENYX PHARMACEUTICAL INC.
		
	By:	 	 

 
			
	Name:	 	
	 Title:
	 	
	
	Address:
	
	PARTICIPANT
	
	 
		
	Print	 	 
		
	Name: 	 	 

 
			
		
	 Address: 
	 	 
		
		 	 

  

			
	ESCROW AGENT:
		
	By:	 	 

 Address: 

  
 C-2-4

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