Document:

Settlement Agreement, dated as of September 2, 2012

 Exhibit 10.1 
 SETTLEMENT AGREEMENT 
 This SETTLEMENT AGREEMENT (this “Agreement”) is
made as of September 2, 2012 between Deutsche Bank AG, London Branch (“Dealer”) and Medicis Pharmaceutical Corporation, a Delaware corporation (“Counterparty”). The term “Counterparty” as used herein
shall include any successor by merger, consolidation or similar transaction. 
 WHEREAS, Dealer and Counterparty are parties to the following
transactions and agreements: 
 (a) The base note hedge transaction (the “Base Note Hedge Transaction”)
evidenced by the letter agreement between Counterparty and Dealer, dated as of May 10, 2012 (the “Base Note Hedge Confirmation”) and the additional note hedge transaction (the “Additional Note Hedge
Transaction” and, together with the Base Note Hedge Transaction, the “Note Hedge Transactions”) evidenced by the letter agreement between Counterparty and Dealer, dated as of May 11, 2012 (the “Additional Note
Hedge Confirmation” and, together with the Base Note Hedge Confirmation, the “Note Hedge Confirmations”), each relating to the 1.375% Convertible Senior Notes of Counterparty due June 1, 2017 (the
“Notes”). 
 (b) The base warrant transaction (the “Base Warrant Transaction”) evidenced by the
letter agreement between Counterparty and Dealer, dated as of May 10, 2012 (the “Base Warrant Confirmation”) and the additional warrant transaction (the “Additional Warrant Transaction” and, together with the
Base Warrant Transaction, the “Warrant Transactions”) evidenced by the letter agreement between Counterparty and Dealer, dated as of May 11, 2012 (the “Additional Warrant Confirmation” and, together with the
Base Warrant Confirmation, the “Warrant Confirmations”). 
 WHEREAS, Counterparty or a proposed purchaser of Counterparty (a
“Buyer”) intends to announce the sale, pursuant to a negotiated agreement between Buyer and Counterparty providing for a one-step merger, of all of the outstanding Class A common stock, par value $0.014 per share (the
“Shares”), of Counterparty to Buyer for cash at a price equal to or greater than US$44.00 (as adjusted for any stock split, stock dividend, stock combination or similar event) per Share (the “Specified Price”) (such
cash sale at a price per Share equal to or greater than the Specified Price, a “Merger” and each such announcement of a Merger, an “Announcement”); 
 WHEREAS, Counterparty and Deutsche Bank Securities Inc., an affiliate of Dealer (“DBSI”), have entered into an Engagement Letter, dated as of December 22, 2011 (as amended effective
as of June 19, 2012 and as it may be amended in the future, the “Engagement Letter”), pursuant to which DBSI is acting as financial advisor to Counterparty in connection with, among other things, a potential Merger; 

WHEREAS, the parties have agreed to settle each of (a) the Note Hedge Transactions and the Warrant Transactions (together, the
“Transactions”), and (b) the Note Hedge Confirmations and the Warrant Confirmations (together, the “Confirmations”), in each case, on the terms and conditions set forth herein, in connection with the
consummation of a Merger. 
 NOW, THEREFORE, in consideration of their mutual covenants contained herein and for other good and valuable
consideration, the receipt and sufficiency of which are hereby acknowledged, the parties hereto, intending to be legally bound, hereby mutually covenant and agree as follows: 
 1. Settlement of Transactions and Confirmations. Subject to Section 3 below, immediately upon the Public Announcement (as defined below) that a Merger has closed: 

(a) each Transaction and each Confirmation, and the respective rights and obligations of each party hereto under each Transaction and each
Confirmation, shall be deemed to have been satisfied and discharged in full; 
 (b) each party hereto shall be released and
forever discharged by each other party hereto from the Transactions and the Confirmations; and 

 (c) each party hereto irrevocably, knowingly, and voluntarily releases, waives, and forever
discharges, and agrees not to assert, any and all claims, demands, actions, or causes of action, of any kind whatsoever, known or unknown, foreseen or unforeseen, foreseeable or unforeseeable, which it has or may have, under any source of law or
equity or any other source, against any other party hereto or against any other party’s predecessors, subsidiaries, divisions, affiliates, successors and assigns, and current and former directors, officers, agents and employees
(i) relating to the Transactions or the Confirmations or (ii) with respect to any obligations or liabilities of any other party hereto arising out of and to be performed in connection with the Transactions or the Confirmations, 

in each case, other than (A) in respect of the payment of the Settlement Amount pursuant to Section 2 below, and (B) for the avoidance of
doubt, any payments, rights or claims owing to, or inuring to the benefit of, Dealer or its affiliates (1) as holders of the Notes or of the Shares or (2) in respect of the Engagement Letter (including fees and indemnities payable
thereunder). The satisfaction, release and discharge set forth in this Section 1 shall apply regardless of whether any Transaction or Confirmation is, or is purported to be, terminated, exercised or cancelled concurrently with or prior to
payment of the Settlement Amount pursuant to Section 2 below. For the avoidance of doubt, upon the satisfaction, release and discharge as set forth above, the Transactions and the Confirmations shall terminate and Counterparty’s sole
remedy for Dealer’s failure to pay the Settlement Amount (as set forth below) shall be pursuant to this Agreement and not pursuant to any Transaction or Confirmation. For purposes of this Agreement, “Public Announcement” means
the disclosure by Counterparty or Buyer or their respective affiliates in a press release reported by the Dow Jones News Service, the Business Wire, the Associated Press, Bloomberg or a comparable national news service or in a document publicly
filed by Counterparty or Buyer or their respective affiliates with the Securities and Exchange Commission pursuant to the Securities Exchange Act of 1934, as amended, or the rules and regulations thereunder or in a notice pursuant to the applicable
rules of an exchange on which the securities of Counterparty are listed. 
 2. Settlement Payment. Subject to Section 3 below, in
lieu of (a) Dealer’s paying or delivering to Counterparty any amount otherwise due to Counterparty under the Note Hedge Confirmations with respect to the Relevant Note Hedging Units (as defined below) and (b) Counterparty’s
paying or delivering to Dealer any amount otherwise due to Dealer under the Warrant Confirmations with respect to the Relevant Warrants (as defined below), Dealer shall pay to Counterparty on the Payment Date (as defined below) an aggregate amount
(the “Settlement Amount”) equal to (i) the product of (x) the Note Hedging Unit Amount per Relevant Note Hedging Unit (as specified in Annex A) and (y) the number of Relevant Note Hedging Units, minus
(ii) the product of (x) the Warrant Amount per Relevant Warrant (as specified in Annex A) and (y) the number of Relevant Warrants (and in no case shall the Settlement Amount be greater than the Aggregate Net Amount (as specified in
Annex A) in the aggregate for all outstanding and unexercised Note Hedging Units and Warrants as of the date hereof), in each case in full satisfaction of all rights and obligations of the parties hereto arising out of the Transactions and the
Confirmations with respect to the Relevant Note Hedging Units and the Relevant Warrants. 
 The “Relevant Note Hedging
Units” shall consist of each outstanding and unexercised Note Hedging Unit (as defined in the Note Hedge Confirmations) as of the execution of this Agreement (such time, the “Fixing Time”); provided that, if this
Agreement is terminated pursuant to Section 3 below, the Relevant Note Hedging Units shall instead consist of only each such Note Hedging Unit, if any, that is exercised or terminated pursuant to the terms of the Note Hedge Confirmations prior
to the termination of this Agreement (the Note Hedging Units referred to in this proviso collectively, the “Early Unwound Note Hedging Units”). 
 The “Relevant Warrants” shall consist of each of the Relevant Number of outstanding and unexercised Warrants (as defined in the Warrant Confirmations) that have the earliest Expiration
Dates (as defined in the Warrant Confirmations) of any Warrants outstanding and unexercised as of the Fixing Time. 
 The
“Relevant Number” shall equal the product of (i) the number of Relevant Note Hedging Units and (ii) the Note Hedging Unit Entitlement (as defined in the Note Hedge Confirmations). 

The “Payment Date” shall be the business day no later than the third business day following the later to occur of (x)(i)
the Public Announcement of the closing of a Merger or (ii) the termination of this 

  
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Agreement pursuant to Section 3, as applicable, and (y) payment to DBSI or its affiliate of all amounts due under the Engagement Letter (including under Sections 2, 3 and 8 thereof).
The Settlement Amount shall be paid by wire transfer of immediately available funds to the following account: 
  

			
	 Bank:
	  	Wells Fargo Bank, N.A.
	 Swift:
	  	WFBIUS6S
	 ABA/Routing #:
	  	121000248
	 Bank Address:
	  	San Francisco, CA
	 Account Name:
	  	Medicis Pharmaceutical Corp. Operating Account
	 Account #:
	  	6438801379

 3. Termination of Agreement. Unless otherwise agreed by Dealer in writing, this Agreement shall terminate if
(i) an Announcement of a Merger for cash at a price equal to or greater than the Specified Price per Share, regardless of the identity of the Buyer or whether such Announcement is the first Announcement, does not occur on or prior to
September 30, 2012, or (ii) there is no Public Announcement that a Merger has closed on or prior to June 30, 2013, or (iii) an announcement is made that no Merger contemplated by a prior Announcement will be consummated or
Counterparty announces that it no longer desires to consummate a Merger unless such announcement is accompanied by a concurrent announcement that Counterparty has entered into a definitive agreement with respect to a Merger at a price equal to or
greater than the Specified Price per Share, or (iv) DBSI’s engagement has been terminated (other than by DBSI without cause) pursuant to Section 4 of the Engagement Letter, or (v) DBSI does not receive, at or prior to the closing
of the Merger, the fee payable by Counterparty for DBSI’s provision of financial advisory and investment banking services in connection with the Merger and all fees and expenses then due under the Engagement Letter or (vi) any event occurs
that could cause an adjustment to any term of any Transaction or the Notes or could cause a termination or cancellation of any Transaction, other than an Announcement as defined in this Agreement (or the consummation of a Merger). Upon termination
of this Agreement, all of the respective rights and obligations of the parties under this Agreement (other than in respect of the payment of the Settlement Amount pursuant to Section 2 with respect to any Early Unwound Note Hedging Units and
the related Relevant Warrants) shall be cancelled and terminated, in which case each of the Transactions and Confirmations shall continue in full force and effect (other than with respect to any Early Unwound Note Hedging Units and the related
Relevant Warrants). Notwithstanding anything to the contrary herein, if (a)(i) this Agreement has been terminated (other than pursuant to clause (iv) or (v) above) and, subsequent to such termination, Counterparty is considering a
transaction involving the sale of Counterparty or (ii) this Agreement has not been terminated and, under an agreement governing a Merger, the “Outside Date” or “Termination Date” (as defined in such agreement, being,
generally, the date upon which a party who is not in breach may terminate such agreement if a Merger thereunder has not been consummated on or prior to such date) is later than the date set forth in clause (ii) of the first sentence of this
Section 3 and (b) the issues leading to the negotiation of this Agreement have not otherwise been addressed, then, at Counterparty’s request, the parties shall negotiate in good faith to replace this Agreement with a new agreement
substantially on the terms and conditions set forth herein but with such changes as Dealer determines in its commercially reasonable discretion are necessary (including changes to the amount of the Settlement Amount) to preserve the intent of this
Agreement in light of the then prevailing circumstances and market conditions. 
 4. Representations and Warranties. Counterparty
represents and warrants to Dealer, and Dealer represents and warrants to Counterparty, on the date hereof that: 
 (a) it has the
power to execute this Agreement and any other documentation relating to this Agreement to which it is a party, to deliver this Agreement and to perform its obligations under this Agreement, and has taken all necessary action to authorize such
execution, delivery and performance; 
 (b) such execution, delivery and performance do not violate or conflict with any law
applicable to it, any provision of its constitutional documents, any order or judgment of any court or other agency of government applicable to it or any of its assets or any contractual restriction binding on or affecting it or any of its assets;

  
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 (c) all governmental and other consents that are required to have been obtained by it with
respect to this Agreement have been obtained and are in full force and effect, and all conditions of any such consents have been complied with; 
 (d) its obligations under this Agreement constitute its legal, valid and binding obligations, enforceable in accordance with their terms (subject to applicable bankruptcy, reorganization, insolvency,
moratorium or similar laws affecting creditors’ rights generally and subject, as to enforceability, to equitable principles of general application); and 
 (e) (i) it is entering into this Agreement as principal (and not as agent or in any other capacity), (ii) no other party nor any of their agents is acting as a fiduciary for it, (iii) it is not
relying upon any representations except those expressly set forth in this Agreement, (iv) it has not relied on any other party to this Agreement for any legal, regulatory, tax, business, investment, financial, and accounting advice, and it has
made its own investment, hedging, and trading decisions based upon its own judgment and not upon any view expressed by any other party to this Agreement or any of such other party’s agents, and (v) it is entering into this Agreement with a
full understanding of the terms, conditions and risks thereof and it is capable of and willing to assume those risks. 
 5. Notices. Any
notice or other communication in respect of this Agreement shall be given by both e-mail and facsimile transmission to the e-mail address and facsimile number set forth below and will be deemed effective at the earlier of: 

(a) the time that the e-mail is delivered; and 
 (b) the time that the facsimile transmission is received by a responsible employee of the recipient in legible form (it being agreed that the burden of proving receipt will be on the sender and will not
be met by a transmission report generated by the sender’s facsimile machine). 
 For purposes of this Agreement, the addresses for notices
to the parties shall be: 
  

	 	(i)	Counterparty: 

 Medicis
Pharmaceutical Corporation 
 7720 North Dobson Road 
 Scottsdale, Arizona 85256 
 Attention: Richard D. Peterson 

Fax: (480) 291-8847 
 Email: rpeterson@medicis.com 
  

	 	(ii)	Dealer: 

 Deutsche Bank AG,
London Branch 
 c/o Deutsche Bank Securities Inc. 
 60 Wall Street 
 New York, NY 10005 

Attention: Andrew Yaeger 
 Telephone: (212) 250-2717 
 Email: Andrew.Yaeger@db.com 

with a copy to: 
 Deutsche Bank AG, London Branch 
 c/o Deutsche Bank Securities Inc. 

60 Wall Street 

New York, New York 10005 
 Attention: Faiz Khan 
 Telephone: (212) 250-0668 

  
 4 

 Email: Faiz.Khan@db.com 
 6. Disclosure. Notwithstanding anything to the contrary provided in this Agreement or in any of the Confirmations, and notwithstanding any express or implied claims of exclusivity or proprietary
rights, the parties (and each of their employees, representatives or other agents) are authorized to disclose to any and all persons, beginning immediately upon commencement of their discussions and without limitation of any kind, the tax treatment
and tax structure of the Transactions, the Confirmations and this Agreement, and all materials of any kind (including opinions or other tax analyses) that are provided by either party to the other relating to such tax treatment and tax structure.
Counterparty must provide reasonable notice to Dealer prior to any public disclosure of this Agreement, any of the terms hereof or the transaction contemplated hereby, and afford Dealer a reasonable opportunity to comment on such disclosure (such
comments not to be unreasonably refused). 
 7. Matters Related to Agent. The provisions set forth under the caption
“Miscellaneous—Matters Related to Agent” in each of the Confirmations shall survive the satisfaction, release and discharge of the Transactions and Confirmations to the extent provided for in this Agreement. Further, each party agrees
and acknowledges that (i) DBSI acts solely as agent on a disclosed basis with respect to this Agreement, and (ii) DBSI has no obligation, by guaranty, endorsement or otherwise, with respect to the obligations of either Counterparty or
Dealer hereunder. In this regard, each of Counterparty and Dealer acknowledges and agrees to look solely to the other for performance hereunder, and not to DBSI. 
 8. Counterparts. This Agreement may be executed and delivered in any number of counterparts, each of which shall be identical and all of which, taken together, shall constitute one and the same
instrument, and each of the parties hereto may execute this Agreement by signing any such counterpart. 
 9. Amendments. No amendment,
modification or waiver in respect of this Agreement will be effective unless in writing (including a writing evidenced by a facsimile transmission) and executed by each of the parties hereto. 
 10. Entire Agreement. This Agreement constitutes the entire agreement and understanding of the parties with respect to the subject matter and supersedes all prior or contemporaneous written and
oral communications with respect thereto. Each of the parties acknowledges that, in entering into this Agreement, it has not relied on any oral or written representation, warranty or other assurance (except as provided for or referred to in this
Agreement) and waives all rights and remedies which might otherwise be available to it in respect thereof, except that nothing in this Agreement will limit or exclude any liability of a party for fraud. Counterparty agrees that this Agreement and
the transactions contemplated hereby shall be covered by the indemnification and contribution provisions of Section 8 of the Engagement Letter. 
 11. Governing Law; Jurisdiction. THIS AGREEMENT AND ANY CLAIM, CONTROVERSY OR DISPUTE ARISING UNDER OR RELATED TO THIS AGREEMENT SHALL BE GOVERNED BY THE LAWS OF THE STATE OF NEW YORK. THE PARTIES
HERETO IRREVOCABLY SUBMIT TO THE EXCLUSIVE JURISDICTION OF THE COURTS OF THE STATE OF NEW YORK AND THE UNITED STATES COURT FOR THE SOUTHERN DISTRICT OF NEW YORK IN CONNECTION WITH ALL MATTERS RELATING HERETO AND WAIVE ANY OBJECTION TO THE LAYING OF
VENUE IN, AND ANY CLAIM OF INCONVENIENT FORUM WITH RESPECT TO, THESE COURTS. 
 12. Waiver of Jury Trial. EACH PARTY WAIVES, TO THE
FULLEST EXTENT PERMITTED BY APPLICABLE LAW, ANY RIGHT IT MAY HAVE TO A TRIAL BY JURY IN RESPECT OF ANY SUIT, ACTION OR PROCEEDING RELATING TO THIS AGREEMENT. EACH PARTY (I) CERTIFIES THAT NO REPRESENTATIVE, AGENT OR ATTORNEY OF THE OTHER PARTY
HAS REPRESENTED, EXPRESSLY OR OTHERWISE, THAT SUCH OTHER PARTY WOULD NOT, IN THE EVENT OF SUCH A SUIT, ACTION OR PROCEEDING, SEEK TO ENFORCE THE FOREGOING WAIVER AND (II) ACKNOWLEDGES THAT IT AND THE OTHER PARTY HAVE BEEN INDUCED TO ENTER INTO THE
AGREEMENT, AS APPLICABLE, BY, AMONG OTHER THINGS, THE MUTUAL WAIVERS AND CERTIFICATIONS PROVIDED HEREIN. 

  
 5 

 IN WITNESS WHEREOF the parties have executed this Agreement with effect from the date specified on the first
page of this Agreement. 
  

			
	 AGREED:

	
	DEUTSCHE BANK AG, LONDON BRANCH
		
	 By:
	 	 /s/    Andrew Yaeger

	 Name:
	 	Andrew Yaeger
	 Title:
	 	Managing Director
		
	 By:
	 	 /s/    Dushyant Chadha

	 Name:
	 	Dushyant Chadha
	 Title:
	 	Managing Director
	
	 DEUTSCHE BANK SECURITIES INC.
 acting as Agent in connection with the Transactions

		
	 By:
	 	 /s/    Andrew Yaeger

	 Name:
	 	Andrew Yaeger
	 Title:
	 	Managing Director
		
	 By:
	 	 /s/    Dushyant Chadha

	 Name:
	 	Dushyant Chadha
	 Title:
	 	Managing Director

 [Signature page to Settlement Agreement] 

 ACKNOWLEDGED AND CONFIRMED: 
 MEDICIS PHARMACEUTICAL CORPORATION 
  

			
	By:	 	 /s/ Mark Prygocki

	Name:	 	Mark Prygocki
	Title:	 	President

 [Signature page to Settlement Agreement]Employment Agreement

 Exhibit 10.1 
 EMPLOYMENT AGREEMENT 
 THIS EMPLOYMENT AGREEMENT
(“Agreement”) made effective as of September 5, 2012 (“Effective Date”) by and between BioMarin Pharmaceutical Inc., a Delaware corporation (the “Company”) and Jeffrey R. Ajer
(“Employee”). 
 NOW THEREFORE, for good and valuable consideration (the receipt and adequacy of which are
hereby acknowledged and agreed) the parties hereby covenant and agree as follows: 
 1. Title; Duties. The Company hereby
employs the Employee as Senior Vice President and Chief Commercial Officer, to perform such duties consistent with his title and position as may be determined and assigned to him by the Company’s Chief Executive Officer
(“CEO”). The Employee shall be based in Novato, California, provided that Employee acknowledges that his job duties may require significant travel. 
 2. Time and Effort. The Employee agrees to devote substantially all of his professional employment time and effort to the performance of his duties as Senior Vice President and Chief Commercial
Officer of the Company and to perform such other duties consistent with his title and position as are reasonably assigned him from time to time by the CEO. 
 3. Term. The Company agrees to employ the Employee in accordance with the terms of this Agreement, which terms shall be effective commencing on the Effective Date and continuing thereafter until
terminated pursuant to Section 6 or 7 hereof (the “Term” of this Agreement). A review of the Employee’s total compensation will be made by the Board of Directors of the Company (“Board”) at
least annually in or about December of each year based on the overall performance of the Company and the Company’s assessment of the Employee’s contributions to the Company’s performance, although the Board shall not be under any
obligation to make adjustments other than pursuant to its discretion. 
 4. Compensation; Benefits. 

(a) Base Salary. For all the services to be rendered by the Employee in any capacity hereunder, including services as an executive
officer, the Company agrees to pay the Employee a base salary (“Base Salary”) of not less than three hundred and sixty-five thousand dollars ($365,000) per annum. Base Salary shall be payable in approximately equal installments in
accordance with the Company’s customary payroll practices. The foregoing annual compensation amount may be, from time to time, adjusted above the Base Salary specified above by action of the Board or appropriate Committee of the Board. In the
event the Base Salary is adjusted upward by the Board, such adjusted amount will be deemed to be the new Base Salary. 
 (b)
Annual Bonus. The Employee shall be entitled to participate in the Company’s generally applicable employee bonus program, with such targets and metrics as may be approved by the Board from time to time. For the 2012 bonus plan,
Employee’s target bonus will be 40% of Base Salary. 
 (c) Benefits Plans. The Employee also shall participate fully
in all insurance, pension, retirement, deferred compensation, stock and stock option, stock purchase or similar compensation and benefit plans and programs pursuant to the terms of such plans or programs. 

 (d) Vacation. The Employee shall be entitled to annual paid vacation time of four
(4) weeks, accruing ratably over the course of each year of employment, to be taken at such time or times as the Employee may select, consistent with his obligations hereunder. Vacation days not taken during an applicable fiscal year may be
carried over to the extent permitted under the Company’s vacation policy to the following fiscal year pursuant such policy. 
 (e) Expenses. The Company shall reimburse the Employee for all reasonable and customary travel, business and entertainment expenses incurred in connection with the Employee’s performance of
his services hereunder in accordance with the policies and procedures established by the Company and paid promptly after the Employee makes a request therefore and no later than the end of the calendar year following the calendar year in which the
expenses were incurred by the Employee. 
 (f) Withholding. The amounts payable pursuant to this Agreement shall be
subject to withholding for appropriate taxes, assessments or withholdings as required by applicable law. 
 5. Other
Plans. The Company and the Employee hereby agree that nothing contained herein is intended to or shall be deemed to affect any of the Employee’s rights as a participant under any retirement, stock option, stock purchase, pension, insurance,
profit-sharing or similar plans of the Company now or hereafter declared to be in effect. The Company recognizes that the Employee is induced to execute this Agreement and to accept compensation at the rate set forth herein in part because he
expects to be a participant under such plans as are, from time to time, in effect for the Company’s executives and/or employees in general. 
 6. Termination for Cause, Resignation Without Good Reason. 
 (a)
Termination for Cause. This Agreement may be terminated for Cause (as defined below) by the Company before the expiration of the Term provided for herein if, during the Term of this Agreement, the Employee (i) materially violates the
provisions of the Non-Competition Agreement or the Confidentiality Agreements between the Company and Employee, (ii) is convicted of, or pleads nolo contendere to, any crime involving misuse or misappropriation of money or other property
of the Company or any felony; (iii) exhibits repeated willful or wanton failure or refusal to perform his duties in furtherance of the Company’s business interest or in accordance with this Agreement, which failure or refusal is not
remedied by the Employee within thirty (30) days after notice from the Company; (iv) commits an intentional tort against the Company, which materially adversely affects the business of the Company; (v) commits any flagrant act of
dishonesty or disloyalty or any act involving gross moral turpitude, which materially adversely affects the business of the Company; or (vi) exhibits immoderate use of alcohol or drugs which, in the opinion of an independent physician selected
by the Company, impairs the Employee’s ability to perform his duties hereunder (all of the foregoing clauses (i) through (vi) constituting reasons for termination for “Cause”), provided that unsatisfactory business
performance of the Company, or mere inefficiency, or good faith errors in judgment or discretion by the Employee shall not constitute grounds for termination for Cause hereunder. In the event of a termination for Cause, the Company may by written
notice immediately terminate his employment and, in that event, the Company shall be obligated only to pay the Employee the compensation due him up to the date of termination, all accrued, vested or earned benefits under any applicable benefit plan
and any other compensation to which the Employee is entitled under Section 4 up to and ending on the date of the Employee’s termination. 
 (b) Resignation Without Good Reason. The benefits and compensation set forth in Section 6(a) are the only compensation and benefits that the Employee will receive in the event that
Employee resigns without Good Reason (as defined below). If the Employee resigns without Good Reason prior to a Change in Control (as defined in the Company’s Change of Control Policy), the

 
Employee agrees to give the Company at least four (4) weeks’ prior notice and in exchange the Company agrees to pay the Employee for all compensation Employee would be entitled to
pursuant to Section 4 for such four (4)-week period as if Employee had not resigned without Good Reason. Any resignation of the Employee hereunder, whether for Good Reason or otherwise, shall be deemed to include a resignation from all
positions and in all capacities with the Company and its subsidiaries, including, without limitation, membership on the boards of directors (and committees thereof) of subsidiaries of the Company, and the Employee shall execute such documentation as
requested by the Company with respect thereto. 
 7. Termination Without Cause; Resignation For Good Reason; Disability;
Death. 
 (a) Termination Without Cause; Resignation For Good Reason. The Company may terminate the Employee’s
employment at any time for any reason or no reason, upon written notice to the Employee. If (i) the Company terminates the Employee’s employment without Cause at any time prior to the end of the Term of the Agreement, or (ii) the
Employee provides the Company with written notice (a “Notice of Termination”) of his resignation for Good Reason (as defined below) at least four (4) weeks prior to the date of termination. 

(b) Resignation for Good Reason. A resignation for any one or more of the following events, without the written consent of
Employee or his approval of such event in his capacity as Vice President, General Counsel, shall be referred to herein as “Good Reason”; 
 (i) a substantial reduction in the Employee’s duties, status, or reporting structure, in either case by reference to the position held by the Employee on the Effective Date; 

(ii) a relocation of the Employee’s assigned office more than fifty (50) miles from its then-current location; 

(iii) any decrease in the Employee’s Base Salary or a material decrease in his Company benefits in the aggregate, other than as
part of a reduction (not exceeding twenty-five percent) that equitably applies to all of the Company’s executive officers; 
 (iv) a material breach of this Agreement by the Company; 
 (c) provided,
however, that an event that is or would constitute grounds for a resignation for Good Reason shall not constitute such grounds for a resignation for Good Reason unless (i) Employee first notifies the Company’s Board of Directors in
writing of the event(s) within ninety (90) days after the initial occurrence of the event, (ii) the Company does not cure such event(s) within thirty (30) days after its receipt of the Employee’s written notice, and
(iii) the Employee does not terminate his employment within thirty (30) days after the expiration of the cure period. Termination Compensation. For purposes of this Agreement, the term “Termination Compensation”
shall mean: (i) one hundred forty percent (140%) of Employee’s then current annual base salary which shall be payable in a lump sum within 2 weeks after separation of employment, conditioned on Employee executing the Company’s
standard form severance and release agreement, and shall be subject to customary withholding and other applicable payroll processes. Employee shall execute the Company’s standard form severance and release agreement within sixty (60) days
after the Employee’s termination. 
 (d) Employee’s Disability. The Company shall be entitled, by providing
written notice to the Employee, to terminate the Employee’s employment under this Agreement if the Employee shall become permanently disabled such that he is unable to carry out his duties hereunder for four (4) consecutive calendar months
or for a period aggregating one hundred twenty (120) days in any period of 

 
twelve (12) consecutive calendar months. If the Employee is eligible to receive benefits under the Company’s Long-Term Disability Plan, then the Company will pay the Employee additional
compensation so that the total received by the Employee (after taking into consideration the amounts payable to the Employee under the Long-Term Disability Plan) equals the Termination Compensation. If the Employee is not eligible to receive
benefits under such plan, then he will upon termination of his employment for permanent disability be entitled to receive the full Termination Compensation. Any delay or forbearance by the Company in exercising any such right to terminate this
Agreement shall not constitute a waiver thereof. 
 (e) Employee’s Death. The Employee’s employment will
immediately terminate upon the death of the Employee. The Employee’s surviving designated beneficiary, or, if none, the Employee’s estate, shall be entitled to receive the compensation due the Employee up to the date of the Employee’s
death, all accrued, vested or earned benefits under any applicable benefit plan and any other compensation to which the Employee is entitled under this Agreement up to and ending on the date of the Employee’s death. 

8. Choice of Law; Venue. This Agreement shall be construed and performed in accordance to the laws (but not the conflicts of laws)
of the State of California. Venue of any proceeding shall be exclusively in the County of Marin in the foregoing state, and both parties consent and agree to such exclusive venue. 

9. Arbitration. 
 (a) The Employee and the Company understand that litigation is a costly and time-consuming process and agree that they will exclusively resolve any disputes between them by binding arbitration. The
Employee and the Company understand that this agreement to arbitrate covers all disputes that the Company may have against the Employee, or that the Employee might have against the Company or its related entities or employees, including those that
relate to the Employee’s employment or termination of employment (for example claims of unlawful discrimination or harassment). 
 (b) The arbitration will be conducted by an impartial arbitrator experienced in employment law (selected from either the JAMS or the American Arbitration Association (at the Company’s election) panel
of arbitrators) in accordance with the applicable entity’s then current employment arbitration rules (except as otherwise provided in this agreement or unless the parties agree to use the then-current commercial arbitration rules). The Employee
and the Company waive the right to institute a court action, except for requests for injunctive relief pending arbitration, and understand that they are giving up their right to a jury trial. The parties shall be entitled to reasonable discovery.
The arbitrator’s award and opinion shall be in writing and in the form typically rendered in labor and employment arbitrations. 
 (c) The Company will pay any filing fee and the fees and costs of the arbitrator, unless the Employee initiates the claim, in which case the Employee only will be required to contribute an amount equal to
the filing fee for a claim initiated in a court of general jurisdiction in the State of California. The Employee and the Company each shall be responsible for their own attorneys’ fees and costs; provided, however, the arbitrator
may award attorneys’ fees to the prevailing party, if permitted by applicable law. This arbitration agreement does not prohibit either the Employee or the Company from filing a claim with an administrative agency (e.g., the EEOC), nor
does it apply to claims for workers’ compensation or unemployment benefits, or claims for benefits under an employee welfare or pension plan that specifies a different dispute resolution procedure. The arbitration shall take place in Marin
County, California unless the Employee and the Company agree otherwise, 

 10. Notices. All notices provided for or permitted to be given pursuant to this
Agreement must be in writing. All notices shall be given to the other party by personal delivery, overnight courier (with receipt signature), or facsimile transmission (with “answerback” confirmation of transmission), to the Company or the
Employee at the Company’s principal executive offices if to the Company or to the residential address of the Employee as contained in Employee’s personnel file if to Employee. Each such notice shall be deemed effective upon the date of
actual receipt in the case of personal delivery, receipt signature in the case of overnight courier, or confirmation of transmission in the case of facsimile. 
 11. Entire Agreement; Amendment. This Agreement contains the sole and entire agreement of the parties and supersedes all prior agreements and understandings between the Employee and the Company and
cannot be modified or changed by any oral or verbal promise or statement by whomsoever made; nor shall any written modification of it be binding upon the Company until such written modification shall have been approved in writing by the CEO or the
Board. 
 12. Waiver; Consent. In the event any term or condition contained in this Agreement should be breached by any
party and thereafter waived or consented to by the other party, which waiver or consent must be effectuated by a written instrument signed by the party against whom any waiver or consent is sought (and, in the case of the Company, approved by the
CEO or the Board), such waiver or consent shall be limited to the particular breach so waived or consented to and shall not be deemed to waive or consent to any other breach occurring prior or subsequent to the breach so waived or consented to.

 13. Severability. If any provisions of this Agreement or the application thereof to any person or circumstances shall
be invalid or unenforceable to any extent, the remainder of this Agreement and the application of such provisions to other persons or circumstances shall not be affected thereby and shall be enforced to the extent permitted by law. 

14. Survival. The provisions hereof which are to be performed or observed after the termination of this Agreement, and the
representations, covenants and agreements of the parties contained herein with respect thereto shall survive the termination of this Agreement and be effective according to their terms. 

15. Successors. All of the terms and provisions of this Agreement shall be binding upon and shall inure to the benefit of and be
enforceable by and against the parties to this Agreement and the respective heirs, executors, and successors in interest; provided, however, that the duties of the Employee hereunder are personal in nature and may not be delegated
without a written consent of the Company. 
 16. Assignment. This Agreement and the rights and benefits contained herein
may not be assigned by either party hereto, except by the Company in connection with a merger, consolidation, share exchange, business combination or other reorganization of the Company or a sale of all or substantially all of the Company’s
business or assets. 
 17. Certain Representations, Covenants and Acknowledgements. 

(a) The Employee represents that he is not subject to any employment, confidentiality, or other agreement or restriction that would
prevent him from fully satisfying his duties under this Agreement or that would be violated if he did so. 
 (b) Without the
Company’s prior written approval, the Employee agrees not to: (i) disclose proprietary information belonging to a former employer or other entity without its written permission; (ii) contact any former employer’s customers or
employees to solicit their business or 

 
employment on behalf of the Company; or (iii) distribute announcements about or otherwise publicize his employment with the Company. 

(c) The Employee acknowledges that he is free to seek advice from independent counsel with respect to this Agreement and the Employee has
obtained such advice. The Employee is not relying on any representation or advice from the Company or any of its officers, directors, attorneys or other representatives regarding this Agreement, its content or effect. 

18. Construction. The masculine pronoun, wherever used herein, shall be construed to include the feminine and the neuter, where
appropriate, The singular form, wherever used herein, shall be construed to include the plural, where appropriate. 
 19.
Drafting. The parties represent and acknowledge that they both have participated in the preparation and drafting of this Agreement and have each given their approval to all of the language contained in this Agreement, and it is expressly
agreed and acknowledged that if either party later claims that there is an ambiguity in the language of this Agreement, there shall be no presumption that such ambiguity be construed for or against either party hereto. 

20. Counterparts. This Agreement may be executed and delivered (including by facsimile transmission) in one or more counterparts,
each of which shall be deemed an original, but all of which together shall constitute one and the same instrument. 
 21.
Compliance with Section 409A the Internal Revenue Code of 1986, as amended. If any amounts or benefits payable under this Agreement on account of Employee’s termination of employment constitute deferred compensation subject to
Section 409A of the Internal Revenue Code of 1986, as amended (the “Code”), no payments or benefits shall be paid or provided until Employee incurs a separation from service within the meaning of Treas. Reg. § 1.409A-1(h) from
the Company and any entity that would be considered a single employer with the Company under Code Sections 414(b) or 414(c) (“Separation from Service”). If, at the time of Employee’s Separation from Service, the Employee is a
“specified employee” (within the meaning of Code Section 409A and Treas. Reg. §1.409A-3(i)(2)), the Company will not pay or provide any “Specified Benefits” (as defined herein) during the six-month period (the
“409A Suspension Period”) beginning immediately after the Employee’s Separation from Service. For purposes of this Agreement, “Specified Benefits” are any amounts or benefits that would be subject to Code Section 409A
penalties if the Company were to pay them, pursuant to this Agreement, on account of the Employee’s Separation from Service. 
 This
Agreement is intended to comply with (or be exempt from) Code Section 409A, and the Company shall have complete discretion to interpret and construe this Agreement and any associated documents in any manner that establishes an exemption from
(or otherwise conforms them to) the requirements of Code Section 409A. If, for any reason including imprecision in drafting, the Agreement does not accurately reflect its intended establishment of an exemption from (or compliance with) Code
Section 409A, as demonstrated by consistent interpretations or other evidence of intent, the provision shall be considered ambiguous and shall be interpreted by the Company in a fashion consistent herewith, as determined in the sole and
absolute discretion of the Company. The Company reserves the right to unilaterally amend this Agreement without the consent of the Employee in order to accurately reflect its correct interpretation and operation, as well as to maintain an exemption
from or compliance with Code Section 409A. Nevertheless, and notwithstanding any other provision of this Agreement, neither the Company nor any of its employees, directors, or their agents shall have any obligation to mitigate, nor to hold the
Employee harmless from, any or all taxes (including any imposed under Code Section 409A) arising under this Agreement. 

 IN WITNESS WHEREOF, the parties to this Agreement have executed this Agreement as of the
date indicated above. 
  

	
	BIOMARIN PHARMACEUTICAL INC.
	By:/s/ Jean-Jacques Bienaimé
	Name: Jean-Jacques Bienaimé
	Its: Chief Executive Officer
	
	EMPLOYEE
	By:/s/ Jeffrey R. Ajer
	Name: Jeffrey R. Ajer

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