Document:

GENTIUM
      S.p.A.

    AMENDED
      AND RESTATED

    2004
      EQUITY INCENTIVE PLAN

    
      
        
        

      

      
        
        

        
          

        

      

       

    

     

    TABLE
      OF CONTENTS

     

    
      	 	 	 	 	
               Page

            
	
              SECTION
                1.

            	 	
              PURPOSE

            	 	
              1

            
	
              SECTION
                2.

            	 	
              DEFINITIONS

            	 	
              1

            
	
              (a)

            	 	
              “ADS”

            	 	
              1

            
	
              (b)

            	 	
              “Affiliate”

            	 	
              1

            
	
              (c)

            	 	
              “Award”

            	 	
              1

            
	
              (d)

            	 	
              “Award
                Agreement”

            	 	
              2

            
	
              (e)

            	 	
              “Board”

            	 	
              2

            
	
              (f)

            	 	
              “Change
                In Control”

            	 	
              2

            
	
              (g)

            	 	
              “Code”

            	 	
              3

            
	
              (h)

            	 	
              “Committee”

            	 	
              3

            
	
              (i)

            	 	
              “Company”

            	 	
              3

            
	
              (j)

            	 	
              “Consultant”

            	 	
              3

            
	
              (k)

            	 	
              “Corporate
                Transaction”

            	 	
              3

            
	
              (l)

            	 	
              “Covered
                Employee”

            	 	
              3

            
	
              (m)

            	 	
              “Director”

            	 	
              3

            
	
              (n)

            	 	
              “Disability”

            	 	
              3

            
	
              (o)

            	 	
              “Employee”

            	 	
              3

            
	
              (p)

            	 	
              “Exchange
                Act”

            	 	
              4

            
	
              (q)

            	 	
              “Exercise
                Price”

            	 	
              4

            
	
              (r)

            	 	
              “Fair
                Market Value”

            	 	
              4

            
	
              (s)

            	 	
              “Grant”

            	 	
              4

            
	
              (t)

            	 	
              “Incentive
                Stock Option” or “ISO”

            	 	
              4

            
	
              (u)

            	 	
              “Key
                Employee”

            	 	
              4

            
	
              (v)

            	 	
              “Non-Employee
                Director”

            	 	
              4

            
	
              (w)

            	 	
              “Non-Employee
                Independent Director”

            	 	
              5

            
	
              (x)

            	 	
              “Nonstatutory
                Stock Option” or “NSO”

            	 	
              5

            
	
              (y)

            	 	
              “Option”

            	 	
              5

            
	
              (z)

            	 	
              “Optionee”

            	 	
              5

            
	
              (aa)

            	 	
              “Parent”

            	 	
              5

            
	
              (bb)

            	 	
              “Participant”

            	 	
              5

            
	
              (cc)

            	 	
              “Plan”

            	 	
              5

            
	
              (dd)

            	 	
              “Restricted
                Stock”

            	 	
              5

            
	
              (ee)

            	 	
              “Restricted
                Stock Agreement”

            	 	
              5

            
	
              (ff)
                

            	 	
              “SAR
                Agreement”

            	 	
              5

            
	
              (gg)

            	 	
              “Securities
                Act”

            	 	
              5

            
	
              (hh)

            	 	
              “Service”

            	 	
              5

            
	
              (ii)

            	 	
              “Share”

            	 	
              5

            
	
              (jj)
                

            	 	
              “Stock
                Appreciation Right” or “SAR”

            	 	
              5

            
	
              (kk)

            	 	
              “Stock
                Bonus”

            	 	
              5

            
	
              (ll)

            	 	
              “Stock
                Bonus Agreement”

            	 	
              5

            
	
              (mm)

            	 	
              “Stock
                Option Agreement”

            	 	
              5

            
	
              (nn)
                

            	 	
              “Stock
                Purchase Right”

            	 	
              6

            
	
              (oo)

            	 	
              “Stock
                Unit”

            	 	
              6

            
	
              (pp)
                

            	 	
              “Stock
                Unit Agreement”

            	 	
              6

            

    

     

    
      
        
        

      

      
        -i-

        
          

        

      

      
        
        

      

    

     

    
      	 	 	 	 	
              Page

            
	
              (qq)
                

            	 	
              “Subsidiary”

            	 	
              6

            
	
              (rr)

            	 	
              “10-Percent
                Shareholder”

            	 	
              6

            
	 	 	 	 	 
	
              SECTION
                3.

            	 	
              ADMINISTRATION

            	 	
              6

            
	
              (a)

            	 	
              Administration
                by Board.

            	 	
              6

            
	
              (b)

            	 	
              Powers
                of Board.

            	 	
              6

            
	
              (c)

            	 	
              Delegation
                to Committee.

            	 	
              7

            
	
              (d)

            	 	
              Effect
                of Board’s Decision.

            	 	
              7

            
	
              (e)

            	 	
              Indemnification.

            	 	
              7

            
	 	 	 	 	 
	
              SECTION
                4.

            	 	
              ELIGIBILITY

            	 	
              8

            
	
              (a)

            	 	
              General
                Rules

            	 	
              8

            
	
              (b)

            	 	
              Incentive
                Stock Options

            	 	
              8

            
	
              (c)

            	 	
              Non-Employee
                Director Options

            	 	
              8

            
	 	 	 	 	 
	
              SECTION
                5.

            	 	
              SHARES
                SUBJECT TO PLAN

            	 	
              9

            
	
              (a)

            	 	
              Basic
                Limitation

            	 	
              9

            
	
              (b)

            	 	
              Additional
                Shares

            	 	
              9

            
	
              (c)

            	 	
              Limits
                on Options and SARs

            	 	
              9

            
	
              (d)

            	 	
              Limits
                on Stock Purchase Rights, Stock Units and Stock Bonuses

            	 	
              9

            
	 	 	 	 	 
	
              SECTION
                6.

            	 	
              TERMS
                AND CONDITIONS OF OPTIONS

            	 	
              10

            
	
              (a)

            	 	
              Stock
                Option Agreement

            	 	
              10

            
	
              (b)

            	 	
              Number
                of Shares

            	 	
              10

            
	
              (c)

            	 	
              Exercise
                Price

            	 	
              10

            
	
              (d)

            	 	
              Exercisability
                and Term

            	 	
              10

            
	
              (e)

            	 	
              Modifications
                or Assumption of Options

            	 	
              10

            
	
              (f)

            	 	
              Transferability
                of Options

            	 	
              11

            
	
              (g)

            	 	
              Restrictions
                on Transfer

            	 	
              11

            
	
              (h)

            	 	
              Incentive
                Stock Option $100,000 Limitation.

            	 	
              11

            
	 	 	 	 	 
	
              SECTION
                7.

            	 	
              PAYMENT
                FOR OPTION SHARES

            	 	
              11

            
	
              (a)

            	 	
              General
                Rule

            	 	
              11

            
	
              (b)

            	 	
              Surrender
                of Stock

            	 	
              11

            
	
              (c)

            	 	
              Cashless
                Exercise

            	 	
              12

            
	
               

            	 	 	 	
               

            
	
              SECTION
                8.

            	 	
              TERMS
                AND CONDITIONS FOR AWARDS OF STOCK PURCHASE RIGHTS AND STOCK
                UNITS.

            	 	
              12

            
	
              (a)

            	 	
              Time,
                Amount and Form of Awards

            	 	
              12

            
	
              (b)

            	 	
              Restricted
                Stock and Stock Unit Agreements

            	 	
              12

            
	
              (c)

            	 	
              Payment
                for Restricted Stock or Stock Unit Awards

            	 	
              12

            
	
              (d)

            	 	
              Form
                and Time of Settlement of Stock Units

            	 	
              12

            
	
              (e)

            	 	
              Vesting
                Conditions

            	 	
              12

            
	
              (f)

            	 	
              Assignment
                or Transfer of Restricted Stock or Stock Units

            	 	
              13

            
	
              (g)

            	 	
              Death
                of Stock Units Recipient

            	 	
              13

            
	
              (h)

            	 	
              Trusts

            	 	
              13

            

    

     

    
      
        
        

      

      
        -ii-

        
          

        

      

      
        
        

      

    

     

    
      	 	 	 	 	 Page
	
              (i)

            	 	
              Voting
                and Dividend Rights

            	 	
              13

            
	
              (j)

            	 	
              Stock
                Units Voting and Dividend Rights

            	 	
              13

            
	
              (k)

            	 	
              Creditors’
                Rights

            	 	
              14

            
	
               

            	 	 	 	
               

            
	
              SECTION
                9.

            	 	
              TERMS
                AND CONDITIONS OF STOCK APPRECIATION RIGHTS

            	 	
              14

            
	
              (a)

            	 	
              SAR
                Agreement

            	 	
              14

            
	
              (b)

            	 	
              Number
                of Shares

            	 	
              14

            
	
              (c)

            	 	
              Exercise
                Price

            	 	
              14

            
	
              (d)

            	 	
              Exercisability
                and Term

            	 	
              14

            
	
              (e)

            	 	
              Exercise
                of SARs

            	 	
              14

            
	
              (f)

            	 	
              Modification
                or Assumption of SAR

            	 	
              15

            
	
               

            	 	 	 	
               

            
	
              SECTION
                10.

            	 	
              TERMS
                AND CONDITIONS OF STOCK BONUSES

            	 	
              15

            
	
              (a)

            	 	
              Stock
                Bonus Agreement.

            	 	
              15

            
	
              (b)

            	 	
              Number
                of Shares.

            	 	
              15

            
	
              (c)

            	 	
              Consideration

            	 	
              15

            
	
              (d)

            	 	
              Vesting.

            	 	
              15

            
	
              (e)

            	 	
              Transferability.

            	 	
              15

            
	
              (f)

            	 	
              Death
                of Stock Bonus Recipient

            	 	
              16

            
	
              (g)

            	 	
              Trusts

            	 	
              16

            
	
              (h)

            	 	
              Stock
                Bonus Voting and Dividend Rights

            	 	
              16

            
	 	 	 	 	 
	
              SECTION
                11.

            	 	
              PROTECTION
                AGAINST DILUTION

            	 	
              16

            
	
              (a)

            	 	
              Adjustments

            	 	
              16

            
	
              (b)

            	 	
              Participant
                Rights

            	 	
              17

            
	 	 	 	 	 
	
              SECTION
                12.

            	 	
              EFFECT
                OF A CORPORATE TRANSACTION

            	 	
              17

            
	
              (a)

            	 	
              Merger
                or Reorganization

            	 	
              17

            
	
              (b)

            	 	
              Acceleration

            	 	
              17

            
	 	 	 	 	 
	
              SECTION
                13.

            	 	
              LIMITATIONS
                ON RIGHTS

            	 	
              17

            
	
              (a)

            	 	
              Retention
                Rights

            	 	
              17

            
	
              (b)

            	 	
              Shareholders’
                Rights

            	 	
              17

            
	
              (c)

            	 	
              Regulatory
                Requirements

            	 	
              17

            
	 	 	 	 	 
	
              SECTION
                14.

            	 	
              WITHHOLDING
                TAXES

            	 	
              18

            
	
              (a)

            	 	
              General

            	 	
              18

            
	
              (b)

            	 	
              Share
                Withholding

            	 	
              18

            
	 	 	 	 	 
	
              SECTION
                15.

            	 	
              DURATION
                AND AMENDMENTS

            	 	
              18

            
	
              (a)

            	 	
              Term
                of the Plan

            	 	
              18

            
	
              (b)

            	 	
              Right
                to Amend, Suspend or Terminate the Plan

            	 	
              18

            
	
              (c)

            	 	
              Right
                to Amend Award.

            	 	
              18

            

    

     

    
      
        
        

      

      
        -iii-

        
          

        

      

      
        
        

      

    

     

    
      	 	 	 	 	 Page
	
              SECTION
                16.

            	 	
              EXECUTION

            	 	
              19

            
	
               

            	 	
              APPENDIX
                A GENTIUM S.P.A. 2004 ITALY STOCK AWARD SUB-PLAN

            	 	
              1

            
	 	 	 	 	 
	
              SECTION
                1.

            	 	
              BACKGROUND
                AND PURPOSE

            	 	
              1

            
	 	 	 	 	 
	
              SECTION
                2.

            	 	
              DEFINITION
                AND CONSTRUCTION

            	 	
              1

            
	 	 	 	 	 
	
              SECTION
                3.

            	 	
              INCORPORATION
                OF PLAN

            	 	
              2

            
	 	 	 	 	 
	
              SECTION
                4.

            	 	
              ITALIAN
                STOCK OPTION TERMS

            	 	
              2

            
	
              (a)

            	 	
              Italian
                Stock Option Agreement

            	 	
              2

            
	
              (b)

            	 	
              Number
                of Shares

            	 	
              3

            
	
              (c)

            	 	
              Exercise
                Price

            	 	
              3

            
	
              (d)

            	 	
              Exercisability
                and Term

            	 	
              3

            
	
              (e)

            	 	
              Withholding
                Obligations.

            	 	
              3

            
	
              (f)

            	 	
              Transferability

            	 	
              3

            
	 	 	 	 	 
	
              SECTION
                5.

            	 	
              ITALIAN
                STOCK GRANT TERMS

            	 	
              3

            
	
              (a)

            	 	
              Italian
                Stock Grant Agreement.

            	 	
              3

            
	
              (b)

            	 	
              Number
                of Shares.

            	 	
              3

            
	
              (c)

            	 	
              Consideration.

            	 	
              3

            
	
              (d)

            	 	
              Vesting

            	 	
              4

            
	
              (e)

            	 	
              Withholding
                Obligations

            	 	
              4

            
	
              (f)

            	 	
              Transferability

            	 	
              4

            
	 	 	 	 	 
	
              SECTION
                6.

            	 	
              EFFECT
                OF A CORPORATE TRANSACTION

            	 	
              4

            
	
              (a)

            	 	
              Merger
                or Reorganization

            	 	
              4

            
	
              (b)

            	 	
              Acceleration

            	 	
              4

            
	 	 	 	 	 
	
              SECTION
                7.

            	 	
              LIMITATIONS
                ON RIGHTS

            	 	
              4

            
	
              (a)

            	 	
              Retention
                Rights

            	 	
              4

            
	
              (b)

            	 	
              Shareholders’
                Rights

            	 	
              4

            
	
              (c)

            	 	
              Regulatory
                Requirements

            	 	
              5

            
	 	 	 	 	 
	
              SECTION
                8.

            	 	
              DURATION
                AND AMENDMENTS

            	 	
              5

            
	
              (a)

            	 	
              Term
                of the Italy Sub-Plan

            	 	
              5

            
	
              (b)

            	 	
              Right
                to Amend, Suspend or Terminate the Italy Sub-Plan

            	 	
              5

            
	
              (c)

            	 	
              Right
                to Amend Italian Award

            	 	
              5

            
	 	 	 	 	 
	
              SECTION
                9.

            	 	
              EXECUTION

            	 	
              5

            

    

     

    
      
        
        

      

      
        -iv-

        
          

        

      

      
        
        

      

    

     

    GENTIUM
      S.p.A.

    AMENDED
      AND RESTATED 

    2004
      EQUITY INCENTIVE PLAN

     

    SECTION
      1. PURPOSE.

     

    The
      Board
      adopted the Gentium S.p.A. 2004 Equity Incentive Plan on March 11, 2005 (the
      “Adoption Date”). The Plan shall become effective as of the date on which Shares
      are first made available to the general public pursuant to an initial public
      offering of the Shares (the “IPO Date”). This Gentium S.p.A. Amended and
      Restated 2004 Equity Incentive Plan (the “Plan”)
      was
      adopted by the Board and became effective on July 31, 2006. 

     

    In
      addition, the Board adopted the Gentium S.p.A. Italy Stock Award Sub-Plan on
      the
      Adoption Date, subject to approval by the Company’s shareholders, which Gentium
      S.p.A. Italy Stock Award Sub-Plan became effective as of the IPO Date. The
      Gentium S.p.A. Amended and Restated Italy Stock Award Sub-Plan (the “Italy
      Sub-Plan) was adopted by the Board and became effective on July 31, 2006. The
      Italy Sub-Plan, attached to the Plan as Appendix A, contains the additional
      provisions that are to be read in conjunction with the Plan and are applicable
      to Italian Key Employees who are liable for income tax in the Republic of
      Italy.

     

    The
      purpose of the Plan is to promote the long-term success of the Company and
      the
      creation of shareholder value by offering Key Employees an opportunity to
      acquire a proprietary interest in the success of the Company, or to increase
      such interest, and to encourage such selected persons to continue to provide
      services to the Company and to attract new individuals with outstanding
      qualifications.

     

    The
      Plan
      seeks to achieve this purpose by providing for Awards in the form of Stock
      Purchase Rights granting Restricted Stock, Stock Units, Stock Bonuses and
      Options (which may constitute Incentive Stock Options or Nonstatutory Stock
      Options).

     

    The
      Plan
      shall be governed by, and construed in accordance with, the laws of the Republic
      of Italy. Capitalized terms shall have the meaning provided in Section 2 of
      the
      Plan or the Italy Sub-Plan unless otherwise provided in this Plan or the
      applicable Award Agreement, or other applicable agreement. 

     

    On
      July
      31, 2006, the Board resolved upon certain amendments to the Plan, and adopted
      the text reported hereinbelow. 

     

    SECTION
      2. DEFINITIONS.

     

    (a) “ADS”
means
      American
      Depositary Shares issued pursuant to the Deposit Agreement dated June 15, 2005
      between the Company and Bank of New York, each ADS representing one Ordinary
      Share.

    
      
        
        

      

      
        
        

        
          

        

      

       

    

     

    (b) “Affiliate”
means
      any entity other than a Subsidiary, if the Company and/or one or more
      Subsidiaries own not less than 50% of such entity. For purposes of determining
      an individual’s “Service,” this definition shall include any entity other than a
      Subsidiary, if the Company, a Parent and/or one or more Subsidiaries own not
      less than 50% of such entity.

     

    (c) “Award”
means
      any Grant of an Option, SAR, Stock Unit, Stock Bonus or Stock Purchase Right
      under the Plan.

     

    (d) “Award
      Agreement”
means
      a
      written agreement between the Company and a Participant evidencing the terms
      and
      conditions of a Grant of an individual Award. Each Award Agreement shall be
      subject to the terms and conditions of the Plan.

     

    (e) “Board”
means
      the Board of Directors of the Company, as constituted from time to
      time.

     

    (f) “Change
      In Control”
except
      as may otherwise be provided in an Award Agreement or other applicable
      agreement, means the occurrence of any of the following:

     

    (i) The
      consummation of a merger or consolidation of the Company with or into another
      entity or any other corporate reorganization, if more than 50% of the combined
      voting power of the continuing or surviving entity’s securities outstanding
      immediately after such merger, consolidation or other reorganization is owned
      by
      persons who were not shareholders of the Company immediately prior to such
      merger, consolidation or other reorganization;

     

    (ii) The
      sale,
      transfer or other disposition of all or substantially all of the Company’s
      assets;

     

    (iii) Any
      transaction as a result of which any person becomes the beneficiary, owner
      and/or “beneficial owner” (as defined in Rule 13d-3 under the Exchange Act),
      directly or indirectly, of securities of the Company representing at least
      20%
      of the total voting power represented by the Company’s then outstanding voting
      securities. For purposes of this Paragraph (iii), the term “person” shall
      indicate any person and/or entity except for:

     

    (A) A
      trustee
      or other fiduciary holding securities under an employee benefit plan of the
      Company or a subsidiary of the Company;

     

    (B) A
      corporation owned directly or indirectly by the shareholders of the Company
      in
      substantially the same proportions as their ownership of ordinary shares of
      the
      Company; and

     

    (C) The
      Company; or

     

    (iv) A
      complete liquidation or dissolution of the Company.

     

    (g) “Code”
means
      the United States Internal Revenue Code of 1986, as amended.

    
      
        
        

      

      
        2

        
          

        

      

       

    

     

    (h) “Committee”
means
      a
      committee consisting of one or more members of the Board that is appointed
      by
      the Board (as described in Section 3) to administer the Plan.

     

    (i) “Company”
means
      Gentium S.p.A., a stock corporation organized under the laws of the Republic
      of
      Italy.

     

    (j) “Consultant”
means
      an individual who performs bona fide services to the Company, a Parent, a
      Subsidiary or an Affiliate other than as an Employee or Director or Non-Employee
      Director.

     

    (k) “Corporate
      Transaction”
means
      the occurrence, in a single transaction or in a series of related transactions,
      of any one or more of the following events:

     

    (i) a
      sale or
      other
      disposition of all or substantially all, as determined by the Board in its
      discretion, of the consolidated assets of the Company and its
      Subsidiaries;

     

    (ii) a
      merger,
      consolidation or similar transaction following which the Company is not the
      surviving corporation; or

     

    (iii) a
      merger,
      consolidation or similar transaction following which the Company is the
      surviving corporation but the outstanding shares thereof immediately preceding
      the merger, consolidation or similar transaction are converted or exchanged
      by
      virtue of the merger, consolidation or similar transaction into other property,
      whether in the form of securities, cash or otherwise.

     

    (l) “Covered
      Employee”
      means
      the chief executive officer, and the 4 (four) other highest compensated officers
      of the Company.

     

    (m) “Director”
means
      a
      member of the Board who is also an Employee.

     

    (n) “Disability”
means
      that the Key Employee is unable to engage in any substantial gainful activity
      by
      reason of any medically determinable physical or mental impairment which can
      be
      expected to result in death or which has lasted or can be expected to last
      for a
      continuous period of not less than 12 months.

     

    (o) “Employee”
means
      any individual who is an employee of the Company, a Parent, a Subsidiary or
      an
      Affiliate. Mere service as a Director or payment of a director’s fee by the
      Company or an Affiliate shall not be sufficient to constitute “employment” by
      the Company or an Affiliate.

     

    (p) “Exchange
      Act”
means
      the United States Securities Exchange Act of 1934, as amended.

     

    (q) “Exercise
      Price”
means,
      in the case of an Option, the amount for which a Share may be purchased upon
      exercise of such Option, as specified in the applicable Stock Option Agreement.
      “Exercise Price,” in the case of a SAR, means an amount, as specified in the
      applicable SAR Agreement, which is subtracted from the Fair Market Value of
      a
      Share in determining the amount payable upon exercise of such
      SAR.

    
      
        
        

      

      
        3

        
          

        

      

       

    

     

    (r) “Fair
      Market Value”
means
      the market price of Shares, determined by the Committee as follows:

     

    (i) If
      ADSs
      were traded on a stock exchange on the date in question, then the Fair Market
      Value shall be equal to the closing price reported by the applicable composite
      transactions report for such date;

     

    (ii) If
      the
      ADSs were traded over-the-counter on the date in question and were classified
      as
      a national market issue or small cap issue, then the Fair Market Value shall
      be
      equal to the closing price quoted by the NASDAQ system for such
      date;

     

    (iii) If
      the
      ADSs were traded over-the-counter on the date in question but were not
      classified as a national market issue, then the Fair Market Value shall be
      equal
      to the mean between the last reported representative bid and asked prices quoted
      by the applicable trading market for such date; and

     

    (iv) If
      none
      of the foregoing provisions is applicable, then the Fair Market Value shall
      be
      determined by the Committee in good faith on such basis as it deems
      appropriate.

     

    Whenever
      possible, the determination of Fair Market Value by the Committee shall be
      based
      on the prices reported in the Wall
      Street Journal.
      Such
      determination shall be conclusive and binding on all persons. It remains
      understood that, pursuant to the resolution adopted by the Extraordinary
      Shareholders’ Meeting of the Company on September 30, 2004, the Exercise Price
      shall not be lower than an amount corresponding to Euro 4.50. 

     

    (s) “Grant”
means
      any grant of an Award under the Plan.

     

    (t) “Incentive
      Stock Option”
or
      “ISO”
means
      an incentive stock option described in Code section 422(b).

     

    (u) “Key
      Employee”
means
      an Employee, Director, Non-Employee Director or Consultant who has been selected
      by the Committee to receive an Award under the Plan.

     

    (v) “Non-Employee
      Director”
means
      a
      member of the Board who is not an Employee.

     

    (w) “Non-Employee
      Independent Director”
means
      a
      Non-Employee Director who: 

     

    
      	
            	(i)	
              is
                not a current Employee, is not a former Employee who received compensation
                for prior services in the current year, and has not been an officer
                of the
                Company; 

            

    

    
      
        
        

      

      
        4

        
          

        

      

       

    

     

    
      	
            	(ii)	
              did
                not directly or indirectly receive more than $60,000 in remuneration
                from
                the Company during the current year, other than director fees;
                and

            

    

     

    
      	
            	(iii)	
              did
                not receive more than $60,000 in remuneration from the Company during
                the
                prior year, other than director
                fees.

            

    

     

    (x)  “Nonstatutory
      Stock Option”
or
      “NSO”
means
      a
      stock option that is not an ISO.

     

    (y) “Option”
means
      an ISO or NSO granted under the Plan entitling the Optionee to purchase
      Shares.

     

    (z) “Optionee”
means
      an individual, estate or other entity that holds an Option.

     

    (aa) “Parent”
means
      a
“parent corporation,” whether now or hereafter existing, as defined in
      section 424(e) of the Code.

     

    (bb) “Participant”
      means an
      individual or estate or other entity that holds an Award.

     

    (cc)
       “Plan”
means
      this Gentium S.p.A. Amended and Restated 2004 Equity Incentive Plan as it may
      be
      amended from time to time.

     

    (dd)  “Restricted
      Stock”
      means a
      Share awarded under the Plan pursuant to a Stock Purchase Right.

     

     (ee)
       “Restricted
      Stock Agreement”
      means
      the agreement described in Section 8 evidencing Restricted Stock that may
      be purchased following the Grant of a Stock Purchase Right.

     

    (ff)
       “SAR
      Agreement”
means
      the agreement described in Section 9 evidencing a Grant of a Stock Appreciation
      Right.

     

    (gg) “Securities
      Act”
means
      the United States Securities Act of 1933, as amended.

     

    (hh)
       “Service”
means
      service as an Employee, Director, Non-Employee Director or Consultant. A change
      in the capacity in which the Participant renders service to the Company or
      an
      Affiliate as an Employee, Consultant, Director or Non-Employee Director or
      a
      change in the entity for which the Participant renders such service, provided
      that there is no interruption or termination of the Participant’s service with
      the Company or an Affiliate, shall not terminate a Participant’s Service. For
      example, a change in status from an Employee of the Company to a Consultant
      of
      an Affiliate or a Director shall not constitute an interruption of
      Service.

     

    (ii)
      “Share”
means
      one ordinary share of the Company.

     

    (jj)
      “Stock
      Appreciation Right” or “SAR”
      means
      a stock
      appreciation right awarded under the Plan.

     

    (kk) “Stock
      Bonus”
      means a
      stock bonus awarded under the Plan.

    
      
        
        

      

      
        5

        
          

        

      

       

    

     

    (ll)“Stock
      Bonus Agreement”
      means
      the agreement described in Section 9 evidencing each Grant of a Stock
      Bonus.

     

    (mm)
      “Stock
      Option Agreement”
means
      the agreement described in Section 6 evidencing each Grant of an
      Option.

     

    (nn)
      “Stock
      Purchase Right”
means
      the right to acquire Restricted Stock pursuant to Section 8.

     

    (oo)
      “Stock
      Unit”
means
      a
      bookkeeping entry representing the equivalent of a Share, as awarded under
      the
      Plan.

     

    (pp)
      “Stock
      Unit Agreement”
means
      the agreement described in Section 8 evidencing a Grant of Stock
      Units.

     

    (qq)
      “Subsidiary”
means
      a
“subsidiary corporation,” whether now or hereafter existing, as defined in
      section 424(f) of the Code.

     

    (rr)
      “10-Percent
      Shareholder”
means
      an individual who owns more than ten percent (10%) of the total combined voting
      power of all classes of outstanding stock of the Company, its Parent or any
      of
      its Subsidiaries. In determining stock ownership, the attribution rules of
      section 424(d) of the Code shall be applied.

     

    SECTION
      3. ADMINISTRATION.

     

    (a) Administration
      by Board.
      The
      Board shall administer the Plan and the Italy Sub-Plan unless and until the
      Board delegates administration to a Committee, as provided in Section
      3(c).

     

    (b) Powers
      of Board.
      The
      Board shall have the power, subject to, and within the limitations of, the
      express provisions of the Plan and Italy Sub-Plan:

     

    (i) to
      determine from time to time which of the persons eligible under the Plan or
      the
      Italy Sub-Plan shall be granted Awards or Italian Awards, as applicable; when
      and how each Award or Italian Award shall be granted; what type or combination
      of types of Award or Italian Awards shall be granted; the provisions of each
      Award or Italian Awards granted (which need not be identical), including the
      time or times when a person shall be permitted to receive Shares pursuant to
      a
      Award or Italian Award; and the number of Shares with respect to which an Award
      or Italian Award shall be granted to each such person;

     

    (ii) to
      construe and interpret the Plan, Italy Sub-Plan, Awards and Italian Awards
      granted under them, and to establish, amend and revoke rules and regulations
      for
      its administration. The Board, in the exercise of this power, may correct any
      defect, omission or inconsistency in the Plan or the Italy Sub-Plan or in any
      Award Agreement or Italian Award Agreement, in a manner and to the extent it
      shall deem necessary or expedient to make the Plan and Italy Sub-Plan fully
      effective;

    
      
        
        

      

      
        6

        
          

        

      

       

    

     

    (iii) to
      amend
      the Plan or an Award as provided in Section 15;

     

    (iv) to
      terminate or suspend the Plan as provided in Section 15;
      and

     

    (v) generally,
      to exercise such powers and to perform such acts as the Board deems necessary
      or
      expedient to promote the best interests of the Company which are not in conflict
      with the provisions of the Plan or Italy Sub-Plan.

     

    (c) Delegation
      to Committee.

     

    (i) The
      Board
      may delegate administration of the Plan to a Committee or Committees of the
      Board, and the term “Committee” shall apply to persons to whom such authority
      has been delegated. 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 (and references in this Plan to the Board
      shall thereafter be to the Committee), subject, however, to such resolutions,
      not inconsistent with the provisions of the Plan, as may be adopted from time
      to
      time by the Board. The Board may abolish the Committee at any time and revest
      in
      the Board the administration of the Plan.

     

    (ii) With
      respect to Awards granted to any Covered Employee, the Committee shall consist
      of those individuals who are
      outside Non-Employee Independent Directors. 

     

    The
      Board
      may also appoint one or more separate committees of the Board, each composed
      of
      directors of the Company who need not to be outside Non-Employee Independent
      Directors..

     

    Notwithstanding
      the foregoing, the Board shall constitute the Committee and shall administer
      the
      Plan with respect to all Awards granted to Non-Employee Directors.

     

    (d) Effect
      of Board’s Decision.
      All
      determinations, interpretations and constructions made by the Board are not
      subject to review by any person and shall be final, binding and conclusive
      on
      all persons.

     

    (e) Indemnification.
      Each
      member of the Committee, or of the Board, shall be indemnified and held harmless
      by the Company against and from (i) any loss, cost, liability, or expense
      that may be imposed upon or reasonably incurred by him or her 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 taken
      or failure to act under the Plan or Italy Sub-Plan or any Award Agreement or
      Italian Award Agreement, and (ii) from any and all amounts paid by him or
      her in settlement thereof, with the Company’s approval, or paid by him or her in
      satisfaction of any judgment in any such claim, action, suit, or proceeding
      against him or her, provided he or she shall give 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, except for the case of fraud
      or
      gross negligence. The foregoing right of indemnification shall not be exclusive
      of any other rights of indemnification to which such persons may be entitled
      under the Company’s Certificate of
      Incorporation or Bylaws, by contract, as a matter of law, or otherwise, or
      under
      any power that the Company may have to indemnify them or hold them
      harmless.

    
      
        
        

      

      
        7

        
          

        

      

       

    

     

    SECTION
      4. ELIGIBILITY.

     

    (a) General
      Rules.
      Only
      Employees, Directors, Non-Employee Directors and Consultants who are liable
      for
      income tax in the United States shall be eligible for designation as Key
      Employees by the Committee.

     

    (b) Incentive
      Stock Options.
      Only
      Key Employees shall be eligible for the grant of ISOs. In addition, a Key
      Employee who is a 10-Percent Shareholder shall not be eligible for the grant
      of
      an ISO unless the requirements set forth in Sections
      6(c) and 6(d) with respect to ISOs.

     

    (c) Non-Employee
      Director Options.
      Non-Employee Directors shall also be eligible to receive Options as described
      in
      this Section 4(c) from and after the date the Board has determined to implement
      this provision.

     

    (i) Each
      eligible Non-Employee Director elected or appointed after the IPO Date shall
      automatically be granted an NSO to purchase 10,000 Shares (subject to adjustment
      under Section 11(a)) as a result of his or her initial election or appointment
      as a Non-Employee Director. All NSOs granted pursuant to this Section 4(c)(i)
      shall vest and become exercisable, provided the individual is serving as a
      Non-Employee Director of the Company as of the vesting date, as follows:
      one-third of the total Shares subject to the NSO (rounded to nearest whole
      number) one year from the date of grant, then in 24 equal monthly installments
      commencing on the date one month and one year after the date of
      grant

     

    (ii) Upon
      the
      conclusion of each regular annual meeting of the Company’s shareholders
      following his or her initial appointment, each eligible Non-Employee Director
      who will continue serving as a member of the Board thereafter shall receive
      an
      NSO to purchase 5,000 Shares (subject to adjustment under Section 11(a)). All
      NSOs granted pursuant to this Section 4(c)(ii) shall vest and become exercisable
      provided the individual is serving as a Non-Employee Director of the Company
      as
      of the vesting date as follows: one-twelfth of the total Shares subject to
      the
      NSO (rounded to nearest whole number) on each monthly anniversary of the date
      of
      grant.

     

    (iii) All
      NSOs
      granted to Non-Employee Directors under this Section 4(c) shall become
      exercisable in full in the event of Change in Control with respect to the
      Company.

     

    (iv) The
      Exercise Price under all NSOs granted to a Non-Employee Director under this
      Section 4(c) shall be equal to one hundred percent (100%) of the Fair Market
      Value of a Share on the date of grant, which shall be payable in one of the
      forms described in Section 7.

     

    (v) All
      NSOs
      granted to a Non-Employee Director under this Section 4(c) shall terminate
      on
      the earlier of:

    
      
        
        

      

      
        8

        
          

        

      

       

    

     

    (A) September
      30, 2009; or

     

    (B) The
      date
      ninety (90) days after the termination of such Non-Employee Director’s Service
      for any reason.

     

    SECTION
      5. SHARES
      SUBJECT TO PLAN.

     

    (a) Basic
      Limitation.
      Subject
      to the provisions of Section 11(a), the stock issuable under the Plan and the
      Italy Sub-Plan shall be authorized but unissued Shares or treasury Shares (the
      latter, within the limits set forth under Italian law). The aggregate number
      of
      Shares reserved for Awards under the Plan and for Italian Awards under the
      Italy
      Sub-Plan shall not exceed 1,500,000 Shares; provided, however, that subject
      to
      the provisions of Section 11(a) of the Plan, the aggregate maximum number of
      Shares that may be issued in connection with ISOs shall be 1,500,000
      Shares. 

     

    (b) Additional
      Shares.
      If
      Awards or Italian Awards are forfeited or terminate for any other reason before
      being exercised, then the Shares underlying such Awards and Italian Awards
      shall
      again become available for Awards under the Plan and Italian Awards under the
      Italy Sub-Plan. 

     

    (c) Limits
      on Options .
      No Key
      Employee shall receive Options to purchase Shares during any fiscal year
      covering in excess of 500,000 Shares. Notwithstanding the foregoing limitation,
      a Key Employee may receive Options to purchase Shares of up to 600,000 Shares
      in
      the first year of a Key Employee’s employment with Company.

     

    (d) Limits
      on Stock Purchase Rights, Stock Units and Stock Bonuses.
      No Key
      Employee shall receive an Award(s) of Stock Purchase Rights, Stock Units and/or
      Stock Bonuses during any fiscal year covering in excess of 250,000 Shares.
      Notwithstanding the foregoing limitation, a Key Employee may receive an Award(s)
      of Stock Purchase Rights, Stock Units and/or Stock Bonuses of up to 500,000
      Shares in the first year of a Key Employee’s employment with
      Company.

     

    SECTION
      6. TERMS
      AND CONDITIONS OF OPTIONS.

     

    (a) Stock
      Option Agreement.
      Each
      Grant of an Option under the Plan shall be evidenced by a Stock Option Agreement
      between the Optionee and the Company. Such Option shall be subject to all
      applicable terms and conditions of the Plan and may be subject to any other
      terms and conditions that are not inconsistent with the Plan and that the
      Committee deems appropriate for inclusion in a Stock Option Agreement. The
      provisions of the various Stock Option Agreements entered into under the Plan
      need not be identical. A Stock Option Agreement may provide that new Options
      will be granted automatically to the Optionee when he or she exercises the
      prior
      Options. The Stock Option Agreement shall also specify whether the Option is
      an
      ISO or an NSO.

     

    (b) Number
      of Shares.
      Each
      Stock Option Agreement shall specify the number of Shares that are subject
      to
      the Option and shall provide for the adjustment of such number in accordance
      with Section 11(a).

    
      
        
        

      

      
        9

        
          

        

      

       

    

     

    (c) Exercise
      Price.
      An
      Option’s Exercise Price shall be established by the Board or the Committee and
      set forth in a Stock Option Agreement. The Exercise Price of an ISO shall not
      be
      less than the higher of (i) 100% of the Fair Market Value (110% for 10-Percent
      Shareholders) of a Share on the date of Grant, and (ii) an amount corresponding,
      as of the date of exercise, to Euro 4.50 per Share. In the case of an NSO,
      a
      Stock Option Agreement may specify an Exercise Price that varies in accordance
      with a predetermined formula while the NSO is outstanding.

     

    (d) Exercisability
      and Term.
      Each
      Stock Option Agreement shall specify the date when all or any installment of
      the
      Option is to become exercisable. The Stock Option Agreement shall also specify
      the term of the Option; provided that the term of an ISO shall in no event
      end
      after September 30, 2009. No Option can be exercised after the expiration date
      provided in the applicable Stock Option Agreement. A Stock Option Agreement
      may
      provide for accelerated exercisability in the event of the Optionee’s death,
      Disability or retirement or other events and may provide for expiration prior
      to
      the end of its term in the event of the termination of the Optionee’s Service. A
      Stock Option Agreement may permit an Optionee to exercise an Option before
      it is
      vested, subject to the Company’s right of repurchase over any Shares acquired
      under the unvested portion of the Option (an “early exercise”), which right of
      repurchase shall lapse at the same rate the Option would have vested had there
      been no early exercise. In no event shall the Company be required to issue
      fractional Shares upon the exercise of an Option.

     

    (e) Modifications
      or Assumption of Options.
      Within
      the limitations of the Plan, the Board or the Committee may modify, extend
      or
      assume outstanding stock options or may accept the cancellation of outstanding
      stock options (whether granted by the Company or by another issuer) in return
      for the grant of new Options for the same or a different number of Shares and
      at
      the same or a different Exercise Price. The foregoing notwithstanding, no
      modification of an Option shall, without the consent of the Optionee, alter
      or
      impair his or her rights or obligations under such Option.

     

    (f) Transferability
      of Options.
      Except
      as otherwise provided in the applicable Stock Option Agreement and then only
      to
      the extent permitted by applicable law, no Option shall be transferable by
      the
      Optionee other than by will or by the laws of descent and distribution. Except
      as otherwise provided in the applicable Stock Option Agreement, an Option may
      be
      exercised during the lifetime of the Optionee only or by the guardian or legal
      representative of the Optionee. No Option or interest therein may be assigned,
      pledged or hypothecated by the Optionee during his or her lifetime, whether
      by
      operation of law or otherwise, or be made subject to execution, attachment
      or
      similar process.

     

    (g) Restrictions
      on Transfer.
      Any
      Shares issued upon exercise of an Option shall be subject to such rights of
      repurchase, rights of first refusal and other transfer restrictions as the
      Committee may determine. Such restrictions shall apply in addition to any
      restrictions that may apply to holders of Shares generally and shall also comply
      to the extent necessary with applicable law.

     

    (h) Incentive
      Stock Option $100,000 Limitation.
      To the
      extent that the aggregate Fair Market Value (determined at the time of grant)
      of
      the Shares with respect to which ISOs are exercisable for the first time by
      any
      Optionee during any calendar year (under all plans of the Company and its
      Affiliates) exceeds one hundred thousand dollars ($100,000), the Options or
      portions thereof which exceed such limit (according to the order in which they
      were granted) shall be treated as NSOs.

    
      
        
        

      

      
        10

        
          

        

      

       

    

     

    SECTION
      7. PAYMENT
      FOR OPTION SHARES.

     

    General
      Rule.
      The
      entire Exercise Price of Shares issued upon exercise of Options shall be paid
      in
      cash at the time when such Shares are purchased.

     

    SECTION
      8. TERMS
      AND CONDITIONS FOR AWARDS OF STOCK PURCHASE RIGHTS AND STOCK
      UNITS.

     

    (a) Time,
      Amount and Form of Awards.
      Awards
      under this Section 8 may be granted in the form of Stock Purchase Rights, in
      the
      form of Stock Units, or in any combination of both. Such Awards may also be
      awarded in combination with NSOs or SARs, and such an Award may provide that
      the
      Restricted Stock or Stock Units will be forfeited in the event that the related
      NSOs or SARs are exercised.

     

    (b) Restricted
      Stock and Stock Unit Agreements.
      Each
      Grant of a Stock Purchase Right or Stock Units under the Plan shall be evidenced
      by a Restricted Stock Agreement or Stock Unit Agreement between the Participant
      and the Company. Such Awards shall be subject to all applicable terms and
      conditions of the Plan and may be subject to any other terms and conditions
      that
      are not inconsistent with the Plan and that the Committee deems appropriate
      for
      inclusion in the applicable Award Agreement. The provisions of the various
      Award
      Agreements entered into under the Plan need not be identical.

     

    (c) Payment
      for Restricted Stock or Stock Unit Awards.
      Restricted Stock or Stock Units shall be issued with cash consideration (which
      in no event will be lower than Euro 4.50 per share) under the Plan.

     

    (d) Form
      and Time of Settlement of Stock Units.
      Settlement of vested Stock Units shall be made in cash. The actual number of
      Stock Units eligible for settlement may be larger or smaller than the number
      included in the original Award, based on predetermined performance factors.
      Methods of converting Stock Units into cash may include (without limitation)
      a
      method based on the average Fair Market Value of Shares over a series of trading
      days. The distribution may occur or commence when all vesting conditions
      applicable to the Stock Units have been satisfied or have lapsed, or it may
      be
      deferred to any later date. The amount of a deferred distribution may be
      increased by an interest factor or by dividend equivalents. Until an Grant
      of
      Stock Units is settled, the number of such Stock Units shall be subject to
      adjustment pursuant to Section 11(a).

     

    (e) Vesting
      Conditions.
      Each
      Grant of Restricted Stock or Stock Units shall become vested upon satisfaction
      of the conditions specified in the applicable Award Agreement. An Award
      Agreement may provide for accelerated vesting in the event of the Participant’s
      death, Disability, retirement or other events.

    
      
        
        

      

      
        11

        
          

        

      

       

    

     

    (f) Assignment
      or Transfer of Restricted Stock or Stock Units.
      Except
      as provided in Section 14, or in a Restricted Stock Agreement or Stock Unit
      Agreement, or as required by applicable law, a Restricted Stock or Stock Unit
      Award granted under the Plan shall not be anticipated, assigned, attached,
      garnished, optioned, transferred or made subject to any creditor’s process,
      whether voluntarily, involuntarily or by operation of law. Any act in violation
      of this Section 8(f) shall be void. However, this Section 8(f) shall not
      preclude a Participant from designating a beneficiary who will receive any
      outstanding Restricted Stock or Stock Unit Awards in the event of the
      Participant’s death, nor shall it preclude a transfer of Restricted Stock or
      Stock Unit Awards by will or by the laws of descent and
      distribution.

     

    (g) Death
      of Stock Units Recipient.
      Any
      Stock Units Award that becomes payable after the Award recipient’s death shall
      be distributed to the recipient’s beneficiary or beneficiaries. Each recipient
      of a Stock Units Award under the Plan shall designate one or more beneficiaries
      for this purpose by filing the prescribed form with the Company. A beneficiary
      designation may be changed by filing the prescribed form with the Company at
      any
      time before the recipient’s death. If no beneficiary was designated or if no
      designated beneficiary survives the recipient, then any Stock Units Award that
      becomes payable after the recipient’s death shall be distributed to the
      recipient’s estate.

     

    (h) Trusts.
      Neither
      this Section 8 nor any other provision of the Plan shall preclude a Participant
      from transferring or assigning Restricted Stock to (a) the trustee of a trust
      that is revocable by such Participant alone, both at the time of the transfer
      or
      assignment and at all times thereafter prior to such Participant’s death, or (b)
      the trustee of any other trust to the extent approved in advance by the
      Committee in writing. A transfer or assignment of Restricted Stock from such
      trustee to any person other than such Participant shall be permitted only to
      the
      extent approved in advance by the Committee in writing, and Restricted Stock
      held by such trustee shall be subject to all of the conditions and restrictions
      set forth in the Plan and in the applicable Restricted Stock Agreement, as
      if
      such trustee were a party to such Restricted Stock Agreement.

     

    (i) Voting
      and Dividend Rights.
      The
      holders of Restricted Stock awarded under the Plan shall have the same voting,
      dividend and other rights as the Company’s other shareholders. A Restricted
      Stock Agreement, however, may require that the holders of Restricted Stock
      invest any cash dividends received in additional Restricted Stock. Such
      additional Restricted Stock shall be subject to the same conditions and
      restrictions as the Award with respect to which the dividends were paid. Such
      additional Restricted Stock shall not reduce the number of Shares available
      under Section 5.

     

    (j) Stock
      Units Voting and Dividend Rights.
      The
      holders of Stock Units shall have no voting rights. Prior to settlement or
      forfeiture, any Stock Unit awarded under the Plan may, at the Committee’s
      discretion, carry with it a right to dividend equivalents. Such right entitles
      the holder to be credited with an amount equal to all cash dividends paid on
      one
      Share while the Stock Unit is outstanding. Dividend equivalents may be converted
      into additional Stock Units. Settlement of dividend equivalents may be made
      in
      the form of cash, in the form of Shares, or in a combination of both. Prior
      to
      distribution, any dividend equivalents which are not paid shall be subject
      to
      the same conditions and restrictions as the Stock Units to which they
      attach.

    
      
        
        

      

      
        12

        
          

        

      

       

    

     

    (k) Creditors’
      Rights.
      A
      holder of Stock Units shall have no rights other than those of a general
      creditor of the Company. Stock Units represent an unfunded and unsecured
      obligation of the Company, subject to the terms and conditions of the applicable
      Stock Unit Agreement.

     

    SECTION
      9. TERMS
      AND CONDITIONS OF STOCK APPRECIATION RIGHTS.

     

    (a) SAR
      Agreement.
      Each
      Grant of a SAR under the Plan shall be evidenced by a SAR Agreement between
      the
      Award recipient and the Company. Such SAR shall be subject to all applicable
      terms of the Plan and may be subject to any other terms that are not
      inconsistent with the Plan. The provisions of the various SAR Agreements entered
      into under the Plan need not be identical. SARs may be granted in consideration
      of a reduction in the recipient other compensation.

     

    (b) Number
      of Shares.
      Each
      SAR Agreement shall specify the number of Shares to which the SAR pertains
      and
      shall provide for the adjustment of such number in accordance with Section
      11(a).

     

    (c) Exercise
      Price.
      Each
      SAR Agreement shall specify the Exercise Price. A SAR Agreement may specify
      an
      Exercise Price that varies in accordance with a predetermined formula while
      the
      SAR is outstanding.

     

    (d) Exercisability
      and Term.
      Each
      SAR Agreement shall specify the date when all or any installment of the SAR
      is
      to become exercisable. The SAR Agreement shall also specify the term of the
      SAR.
      A SAR Agreement may provide for accelerated exercisability in the event of
      the
      recipient’s death, Disability, retirement or other events and may provide for
      expiration prior to the end of its term in the event of the termination of
      the
      recipient’s Service. SARs may also be awarded in combination with Options,
      Restricted Stock or Stock Units, and such an Award may provide that the SARs
      will not be exercisable unless the related Options, Restricted Stock or Stock
      Units are forfeited. A SAR may be included in an ISO only at the time of Grant
      but may be included in an NSO at the time of Grant or at any subsequent time,
      but not later than six months before the expiration of such NSO. A SAR granted
      under the Plan may provide that it will be exercisable only in the event of
      a
      Change in Control.

     

    (e) Exercise
      of SARs.
      If, on
      the date when a SAR expires, the Exercise Price under such SAR is less than
      the
      Fair Market Value on such date but any portion of such SAR has not been
      exercised or surrendered, then such SAR shall automatically be deemed to be
      exercised as of such date with respect to such portion. Upon exercise of a
      SAR,
      the recipient (or any person having the right to exercise the SAR after his
      or
      her death) shall receive cash from the Company. The amount of cash received
      upon
      exercise of SARs shall, in the aggregate, be equal to the amount by which the
      Fair Market Value (on the date of surrender) of the Shares subject to the SARs
      exceeds the Exercise Price.

    
      
        
        

      

      
        13

        
          

        

      

       

    

     

    (f) Modification
      or Assumption of SARs.
      Within
      the limitations of the Plan, the Committee may modify, extend or assume
      outstanding stock appreciation rights or may accept the cancellation of
      outstanding stock appreciation rights (whether granted by the Company or by
      another issuer) in return for the grant of new SARs for the same or a different
      number of Shares and at the same or a different Exercise Price. The foregoing
      notwithstanding, no modification of a SAR shall, without the consent of the
      Optionee, alter or impair his or her rights or obligations under such
      SAR.

     

    SECTION
      10. TERMS
      AND CONDITIONS OF STOCK BONUSES

     

    (a) Stock
      Bonus Agreement.
      Each
      Grant of a Stock Bonus under the Plan shall be evidenced by a Stock Bonus
      Agreement between the Award recipient and the Company. Such Stock Bonus
      Agreement shall be subject to all applicable terms of the Plan and may be
      subject to any other terms that are not inconsistent with the Plan. The
      provisions of the various Stock Bonus Agreements entered into under the Plan
      need not be identical. 

     

    (b) Number
      of Shares.
      Each
      Stock Bonus Agreement shall specify the number of Shares to which the Stock
      Bonus pertains and shall provide for the adjustment of such number in accordance
      with Section 11(a).

     

    (c) Consideration
      A Stock
      Bonus shall be issued with cash consideration (which in no event will be lower
      than Euro 4.50 per share) under the Plan. A Stock Bonus may be awarded in
      consideration for past services actually rendered to the Company or an Affiliate
      for its benefit.

     

    (d)
      Vesting
      The
      Stock Bonus Agreement shall specify whether the Shares awarded under the Stock
      Bonus Agreement are subject to a share
      repurchase right option in favor of the Company in accordance with a vesting
      schedule to be determined by the Committee, and, in any event, within the limits
      set forth under Italian law. In the event the Service of a recipient of a Stock
      Bonus terminates, the Company may reacquire, within the limits set forth under
      Italian law, any or all of the Shares held by the recipient which have not
      vested as of the date of termination under the terms of the Stock Bonus
      Agreement.

     

    (e)
      Transferability.
      Except
      as provided in Section 10 or Stock Bonus Agreement, or as required by applicable
      law, a Stock Bonus granted under the Plan shall not be anticipated, assigned,
      attached, garnished, optioned, transferred or made subject to any creditor’s
      process, whether voluntarily, involuntarily or by operation of law. Any act
      in
      violation of this Section 10(e) shall be void. However, this Section 10(e)
      shall
      not preclude a recipient of a Stock Bonus from designating a beneficiary who
      will receive any outstanding Stock Bonus in the event of the recipient’s death,
      nor shall it preclude a transfer of a Stock Bonus by will or by the laws of
      descent and distribution.

     

    (f)
      Death
      of Stock Bonus Recipient.
      Any
      Stock Bonus that becomes payable after the recipient’s death shall be
      distributed to the recipient’s beneficiary or beneficiaries. Each recipient of a
      Stock Bonus under the Plan shall designate one or more beneficiaries for this
      purpose by filing the prescribed form with the Company. A beneficiary
      designation may be changed by filing the prescribed form with the Company at
      any
      time before the recipient’s death. If no beneficiary was designated or if no
      designated beneficiary survives the recipient, then any Stock Bonus that becomes
      payable after the recipient’s death shall be distributed to the recipient’s
      estate.

    
      
        
        

      

      
        14

        
          

        

      

       

    

     

    (g)
      Trusts.
      Neither
      this Section 10 nor any other provision of the Plan shall preclude a recipient
      of a Stock Bonus from transferring or assigning the Stock Bonus to (a) the
      trustee of a trust that is revocable by such recipient alone, both at the time
      of the transfer or assignment and at all times thereafter prior to such
      recipient death, or (b) the trustee of any other trust to the extent approved
      in
      advance by the Committee in writing. A transfer or assignment of a Stock Bonus
      from such trustee to any person other than the recipient shall be permitted
      only
      to the extent approved in advance by the Committee in writing, and the Stock
      Bonus held by such trustee shall be subject to all of the conditions and
      restrictions set forth in the Plan and in the applicable Stock Bonus Agreement,
      as if such trustee were a party to such Stock Bonus Agreement.

     

    (h)
      Stock
      Bonus Voting and Dividend Rights.
      The
      holders of Shares deriving from the exercise of a Stock Bonus shall have the
      same voting, dividend and other rights as the Company’s other
      shareholders.

     

    SECTION
      11. PROTECTION
      AGAINST DILUTION.

     

    (a) Adjustments.
      In the
      event of a subdivision of the outstanding Shares, a declaration of a dividend
      payable in Shares, a declaration of a dividend payable in a form other than
      Shares in an amount that has a material effect on the price of Shares, a
      combination or consolidation of the outstanding Shares (by reclassification
      or
      otherwise) into a lesser number of Shares, a recapitalization, reorganization,
      merger, liquidation, spin-off or a similar occurrence (all without the receipt
      of consideration), the Board or the Committee shall make such adjustments as
      it,
      in its reasonable discretion, deems appropriate in order to prevent the dilution
      or enlargement of rights hereunder in one or more of:

     

    (i) the
      number of Shares subject to automatic Grants under Section 4(c); the number
      of
      Shares available for future Awards or Italian Awards under Section 5(a); the
      per
      person Share limits under Sections 5(c) and (d);

     

    (ii) the
      number of Shares covered by each outstanding Award or Italian Award;
      or

     

    (iii) the
      Exercise Price under each outstanding Option or Italian Stock
      Option.

     

    Notwithstanding
      anything in this Plan or the Italy Sub-Plan to the contrary, no adjustment
      shall
      be made with respect to any stock split occurring prior to the IPO
      Date.

     

    (b) Participant
      Rights.
      Except
      as provided in this Section 11, a recipient of an Award or Italian Award
      shall have no rights by reason of any issue by the Company of stock of any
      class
      or securities convertible into stock of any class, any subdivision or
      consolidation of shares of stock of any class, the payment of any stock dividend
      or any other increase or decrease in the number of shares of stock of any
      class.

    
      
        
        

      

      
        15

        
          

        

      

       

    

     

    SECTION
      12. EFFECT
      OF A CORPORATE TRANSACTION.

     

    (a) Merger
      or Reorganization.
      In the
      event that the Company is a party to a Corporate Transaction, outstanding Awards
      shall be subject to the terms and conditions of the agreement memorializing
      such
      Corporate Transaction. Such agreement may provide, without limitation, for
      the
      assumption or substitution of outstanding Awards by the surviving corporation
      or
      its parent, for their continuation by the Company (if the Company is a surviving
      corporation), for accelerated vesting or for their cancellation with or without
      consideration.

     

    (b) Acceleration.
      The
      Committee may determine, at the time of granting an Award, or thereafter, that
      such Award shall become fully exercisable as to all Shares subject to such
      Award
      in the event that a Change in Control occurs with respect to the
      Company.

     

    SECTION
      13. LIMITATIONS
      ON RIGHTS.

     

    (a) Retention
      Rights.
      Neither
      the Plan nor any Award granted under the Plan shall be deemed to give any
      individual a right to remain an Employee, Consultant or Director of the Company.
      The Company reserves the right to terminate the Service of any person at any
      time, and for any reason, subject to applicable laws, the Company’s
      Certificate of
      Incorporation and
      Bylaws and a written employment agreement (if any).

     

    (b) Shareholders’
      Rights.
      A
      Participant shall have no dividend rights, voting rights or other rights as
      a
      stockholder with respect to any Shares covered by his or her Award prior to
      the
      issuance of a stock certificate, or similar means of representations of the
      Shares, for such Shares. No adjustment shall be made for cash dividends or
      other
      rights for which the record date is prior to the date when such certificate
      is
      issued, except as expressly provided in Section 11.

     

    (c) Regulatory
      Requirements.
      Any
      other provision of the Plan notwithstanding, the obligation of the Company
      to
      issue Shares under the Plan shall be subject to all applicable laws, rules
      and
      regulations and such approval by any regulatory body as may be required. The
      Company reserves the right to restrict, in whole or in part, the delivery of
      Shares pursuant to any Award prior to the satisfaction of all legal requirements
      relating to the issuance of such Shares, to their registration, qualification
      or
      listing or to an exemption from registration, qualification or
      listing.

     

    SECTION
      14. WITHHOLDING
      TAXES.

     

    (a) Withholding
      Obligations.
      The
      Company will be entitled to make any applicable withholding or deduction on
      account of any taxes or social security contributions due on benefits derived
      from the Plan pursuant to applicable law. A Participant shall make arrangements
      satisfactory to the Company for the satisfaction of such tax or social security
      obligations that arise in connection with his or her Award. The Company shall
      not be required to issue any Shares or make any cash payment under the Plan
      until such obligations are satisfied.

    
      
        
        

      

      
        16

        
          

        

      

       

    

     

    SECTION
      15. DURATION
      AND AMENDMENTS.

     

    (a) Term
      of the Plan.
      The
      Plan shall become effective on the IPO Date, subject to the approval of the
      Company’s shareholders. No Options shall be exercisable until such shareholder
      approval is obtained. In the event that the shareholders fail to approve the
      Plan within twelve (12) months after its adoption by the Board, any Awards
      made
      shall be null and void and no additional Awards shall be made. The Plan shall
      terminate on September 30, 2009 or on any earlier date pursuant to Section
      15(b)
      of the Plan.

     

    (b) Right
      to Amend, Suspend or Terminate the Plan.
      The
      Board may amend, suspend or terminate the Plan at any time and for any reason.
      The suspension or termination of the Plan, or any amendment thereof, shall
      not
      affect any Award previously granted under the Plan. No Awards shall be granted
      under the Plan after the Plan’s suspension or termination. An amendment of the
      Plan shall be subject to the approval of the Company’s shareholders only to the
      extent required by applicable laws, regulations or rules.

     

    (c) Right
      to Amend Award.
      The
      Board at any time, and from time to time, may amend the terms of any one or
      more
      Awards; provided, however, that the rights under any Award shall not be impaired
      by any such amendment unless (i) the Company requests the consent of the
      Participant and (ii) the Participant consents in writing.

     

    (d) Possible
      Novative Effects. It
      remains understood that the adoption by the Board, during the meeting held
      on
      July 31, 2006, of the amended version of the Plan reported above did not produce
      any novative effect pursuant to sections 1230 and 1235 of the Italian Civil
      Code.

     

    SECTION
      16. EXECUTION.

     

    To
      record
      the adoption of the Plan by the Board, the Company has caused its duly
      authorized officer to execute this Plan on behalf of the Company.

     

    
      	 	 	 
	 	GENTIUM
              S.p.A.
	 
 	 
 	 
 
	 	
              By: 

            	
              /s/ Laura
                Iris Ferro

            
	 	Title: 
	Chairperson,
              Chief Executive Officer &  President

    

     

    
      
        
        

      

      
        17

        
          

        

      

       

    

    

    APPENDIX
      A

    

    GENTIUM
      S.p.A.

    AMENDED
      AND RESTATED

    2004
      ITALY STOCK AWARD SUB-PLAN

    

    SECTION
      1. BACKGROUND
      AND PURPOSE

    

    The
      purpose of this Gentium S.p.A. Amended and Restated 2004 Italy Stock Award
      Sub-Plan (the “Italy Sub-Plan”) is to advance the interest of the Company by
      enabling it and its operating companies to attract and retain the best available
      personnel for positions of substantial responsibility in Italy and to provide
      Italian Key Employees of the Company and its controlling and subsidiary
      companies, with an opportunity for investment in the Company; thereby giving
      them an additional incentive to increase their efforts on behalf of the long
      term success of the Company and its operating companies.

     

    It
      is
      intended that Italian Awards granted under the Italy Sub-Plan shall qualify
      for
      the favorable tax and social security treatment applicable to stock grants
      or
      stock options granted under applicable Italian law and in accordance with the
      relevant provisions set forth by Italian tax law and the Italian tax
      administration. The terms of the Italian Sub-Plan shall be interpreted in
      accordance with the relevant provisions set forth by Italian tax and social
      security laws, as well as the Italian tax and social security administrations.
      For Italian Awards granted under the Italy Sub-Plan, any conflict in terms
      between the Plan and the Italy Sub-Plan shall be governed by the terms in the
      Italy Sub-Plan.

     

    SECTION
      2. DEFINITION
      AND CONSTRUCTION

     

    All
      capitalized terms used in this Italy Sub-Plan shall have the meaning ascribed
      to
      them in either the Italy Sub-Plan or the Plan. Terms not defined in the Italy
      Sub-Plan or the Plan, shall be given their normal and ordinary
      meaning.

     

    (a) “Italian
      Employee” means any individual employed by the Company or a controlling or
      subsidiary company who resides in the Republic of Italy and who is liable for
      income tax in the Republic of Italy.

     

    (b) “Exercise
      Price” means (1) when the Company’s ADSs are traded on a regulated market as
      defined under applicable Italian law, the average of the closing price quoted
      by
      the NASDAQ system over the preceding 30 days and (2) when the Company’s ADSs are
      not on a regulated market as defined under applicable Italian law, the
      proportionate value of the net worth of the Company determined by the Board
      in
      good faith and on such basis as it deems appropriate, taking applicable
      regulations into account.
      It
      remains understood that in no event the Exercise Price will be lower than Euro
      4.50.

    
      
        
        

      

      
        2

        
          

        

      

       

    

     

    (c) “Italy
      Sub-Plan” means the provisions contained herein as Appendix A the Plan to be
      known as the Gentium S.p.A. 2004 Italy Stock Award Sub-Plan, as the same may
      be
      amended from time to time.

     

    (d) “Italian
      Award” means an Italian Stock Option or an Italian Stock Grant.

     

    (e) “Italian
      Award Agreement” means a written agreement between the Company and an Italian
      Key Employee evidencing the terms and conditions of a grant of an individual
      Italian Award. Each Italian Award Agreement shall be subject to the terms and
      conditions of the Italy Sub-Plan.

     

    (f) “Italian
      Key Employee” means an Italian Employee who is liable for income tax in Italy or
      any person eligible to receive an Italian Award under Articles 2349 and 2441
      of
      the Italian Civil Code who is liable for income tax in Italy and who has been
      selected by the Committee to receive an Italian Award under the Italy
      Sub-Plan.

     

    (g) “Italian
      Stock Grant” means a stock grant to acquire Shares pursuant to Article 2349 of
      the Italian Civil Code.

     

    (h) “Italian
      Stock Grant Agreement” means the agreement described in Section 5 of the Italy
      Sub-Plan.

     

    (i) “Italian
      Stock Option” means a stock option to acquire Shares pursuant to Article 2441 of
      the Italian Civil Code.

     

    (j) “Italian
      Stock Option Agreement” means the agreement described in Section 4 of the Italy
      Sub-Plan.

     

    SECTION
      3. INCORPORATION
      OF PLAN

     

    This
      Italy Sub-Plan shall be ancillary to the Plan. The provisions of this Italy
      Sub-Plan shall be applicable only to Italian Key Employees who have or shall
      have a liability for Italian income tax with respect to an Italian Award For
      the
      avoidance of doubt, this Italy Sub-Plan does not apply to any Key Employee
      or
      Participant who does not have Italy income tax liability with respect to their
      Award.

    

    SECTION
      4. ITALIAN
      STOCK OPTION TERMS

     

    (a) Italian
      Stock Option Agreement.
      Each
      grant of an Italian Stock Option under the Italy Sub-Plan shall be evidenced
      by
      an Italian Stock Option Agreement between the Italian Key Employee and the
      Company. Such Italian Stock Option shall be subject to all applicable terms
      and
      conditions of the Italy Sub-Plan and may be subject to any other terms and
      conditions that are not inconsistent with the Italy Sub-Plan and that the Board
      deems appropriate for inclusion in an Italian Stock Option Agreement. The
      provisions of the various Italian Stock Option Agreements entered into under
      the
      Italy Sub-Plan need not be identical.

    
      
        
        

      

      
        3

        
          

        

      

       

    

     

    (b) Number
      of Shares.
      Each
      Italian Stock Option Agreement shall specify the number of Shares that are
      subject to the Italian Stock Option and shall provide for the adjustment of
      such
      number in accordance with Section 11(a) of the Plan. Notwithstanding the
      foregoing, no Italian Key Employee shall receive Italian Stock Options to
      purchase Shares in excess of 10% of the voting rights in the annual meeting
      of
      shareholders or 10% of the capital or equity of the Company.

     

    (c) Exercise
      Price.
      An
      Italian Stock Option’s Exercise Price shall be established by the Board and set
      forth in the Italian Stock Option Agreement.

     

    (d) Exercisability
      and Term.
      Each
      Italian Stock Option Agreement shall specify the date when all or any
      installment of the Italian Stock Option is to become exercisable. The Italian
      Stock Option Agreement shall also specify the term of the Italian Stock Option,
      provided that the Italian Stock Option shall terminate prior to the end of
      its
      term if the Italian Key Employee’s Service with the Company terminates. No
      Italian Stock Option can be exercised after the expiration date provided in
      the
      applicable Italian Stock Option Agreement. 

     

    (e) Withholding
      Obligations. The
      recipient of an Italian Stock Option shall make arrangements satisfactory to
      the
      Company for the satisfaction of any withholding tax obligations that arise
      in
      connection with his or her Italian Stock Option. The Company shall not be
      required to issue any Shares until such obligations are satisfied.

     

    (f) Transferability.
      Except
      as otherwise provided in the applicable Italian Stock Option Agreement and
      then
      only to the extent permitted by applicable law, no Italian Stock Option shall
      be
      transferable by the Italian Key Employee other than by will or by the laws
      of
      descent and distribution. No Italian Stock Option or interest therein may be
      assigned, pledged or otherwise offered as security by the Italian Key Employee
      during his or her lifetime, whether by operation of law or otherwise, or be
      made
      subject to execution, attachment or similar process.

     

    SECTION
      5. ITALIAN
      STOCK GRANT TERMS

     

    (a) Italian
      Stock Grant Agreement.
      Each
      grant of an Italian Stock Grant under the Italy Sub-Plan shall be evidenced
      by
      an Italian Stock Grant Agreement between the Italian Key Employee and the
      Company. Such Italians Stock Grant Agreement shall be subject to all applicable
      terms of the Italy Sub-Plan and may be subject to any other terms that are
      not
      inconsistent with the Italy Sub-Plan. The provisions of the various Italian
      Stock Grant Agreements entered into under the Italy Sub-Plan need not be
      identical. 

     

    (b) Number
      of Shares.
      Each
      Italian Stock Grant Agreement shall specify the number of Shares to which the
      Italian Stock Grant pertains and shall provide for the adjustment of such number
      in accordance with Section 11(a) of the Plan.

     

    (c) Consideration.
      An
      Italian Stock Grant shall be awarded in consideration for past services actually
      rendered to the Company or any of its controlling or subsidiary
      companies.

    
      
        
        

      

      
        4

        
          

        

      

       

    

     

    (d) Vesting
      The
      Shares awarded under an Italian Stock Grant shall be subject to forfeiture
      in
      accordance with a vesting schedule to be determined by the Board.

     

    (e) Withholding
      Obligations The
      recipient of an Italian Stock Grant shall make arrangements satisfactory to
      the
      Company for the satisfaction of any withholding tax obligations that arise
      in
      connection with his or her Italian Stock Grant. The Company shall not be
      required to issue any Shares until such obligations are satisfied.

     

    (f) Transferability
      Except
      as otherwise provided in the applicable Italian Stock Grant Agreement and then
      only to the extent permitted by applicable law, no Italian Stock Grant shall
      be
      transferable by the Italian Key Employee. No Italian Stock Grant or interest
      therein may be assigned, pledged or otherwise offered as security by the Italian
      Key Employee during his lifetime, whether by operation of law or otherwise,
      or
      be made subject to execution, attachment or similar process.

     

    SECTION
      6. EFFECT
      OF A CORPORATE TRANSACTION.

     

    (a) Merger
      or Reorganization.
      In the
      event that the Company is a party to a Corporate Transaction, outstanding
      Italian Awards shall be subject to the terms and conditions of the agreement
      memorializing such Corporate Transaction. Such agreement may provide, without
      limitation, for the assumption or substitution of outstanding Italian Awards
      by
      the surviving corporation or its parent, for their continuation by the Company
      (if the Company is a surviving corporation), for accelerated vesting or for
      their cancellation with or without consideration.

     

    (b) Acceleration.
      The
      Board may determine, at the time of granting an Italian Award, or thereafter,
      that such Italian Award shall become fully exercisable as to all Shares subject
      to such Italian Award in the event that a Change in Control occurs with respect
      to the Company.

     

    SECTION
      7. LIMITATIONS
      ON RIGHTS.

     

    (a) Retention
      Rights.
      Neither
      the Italy Sub-Plan nor any Italian Award granted under the Italy Sub-Plan shall
      be deemed to give any individual a right to remain an employee, consultant
      or
      director of the Company and its controlling and subsidiary companies. The
      Company and its controlling and subsidiary companies reserve the right to
      terminate the Service of any person at any time, and for any reason, subject
      to
      applicable laws, the Company’s Certificate of
      Incorporation and
      Bylaws and a written employment agreement (if any).

     

    (b) Shareholders’
      Rights.
      A
      recipient of an Italian Award shall have no dividend rights, voting rights
      or
      other rights as a shareholder with respect to any Shares covered by his or
      her
      Italian Award prior to the issuance of a stock certificate for such Shares.
      No
      adjustment shall be made for cash dividends or other rights for which the record
      date is prior to the date when such certificate is issued, except as expressly
      provided in Section 11 of the Plan.

    
      
        
        

      

      
        5

        
          

        

      

       

    

     

    (c) Regulatory
      Requirements.
      Any
      other provision of the Italy Sub-Plan notwithstanding, the obligation of the
      Company to issue Shares under the Italy Sub-Plan shall be subject to all
      applicable laws, rules and regulations and such approval by any regulatory
      body
      as may be required. The Company reserves the right to restrict, in whole or
      in
      part, the delivery of Shares pursuant to any Italian Award prior to the
      satisfaction of all legal requirements relating to the issuance of such Shares,
      to their registration, qualification or listing or to an exemption from
      registration, qualification or listing.

     

    SECTION
      8. DURATION
      AND AMENDMENTS.

     

    (a) Term
      of the Italy Sub-Plan.
      The
      Italy Sub-Plan shall become effective on the IPO Date, subject to the approval
      of the Company’s shareholders. No Italian Stock Option shall be exercisable
      until such shareholder approval is obtained. In the event that the shareholders
      fail to approve the Italy Sub-Plan, any Italian Awards made shall be null and
      void and no additional Italian Awards shall be made. The Italy Sub-Plan shall
      terminate on September 30, 2009.

     

    (b) Right
      to Amend, Suspend or Terminate the Italy Sub-Plan.
      The
      Board may amend, suspend or terminate the Italy Sub-Plan at any time and for
      any
      reason. The suspension or termination of the Italy Sub-Plan, or any amendment
      thereof, shall not affect any Italian Award previously granted under the Italy
      Sub-Plan. No Italian Awards shall be granted under the Italy Sub-Plan after
      the
      Italy Sub-Plan’s suspension or termination. An amendment of the Italy Sub-Plan
      shall be subject to the approval of the Company’s shareholders only to the
      extent required by applicable laws, regulations or rules.

     

    (c) Right
      to Amend Italian Award
      The
      Board at any time, and from time to time, may amend the terms of any one or
      more
      Italian Awards; provided, however, that the rights under any Italian Award
      shall
      not be impaired by any such amendment unless (i) the Company requests the
      consent of the recipient of such Italian Award and (ii) the recipient of such
      Italian Award consents in writing.

     

    SECTION
      9. EXECUTION.

     

    To
      record
      the adoption of the Italy Sub-Plan by the Board, the Company has caused its
      duly
      authorized officer to execute this Italy Sub-Plan on behalf of the
      Company.

    
       

      
        	 	 	 
	 	GENTIUM
                S.p.A.
	 
 	 
 	 
 
	 	
                By: 

              	
                /s/ Laura
                  Iris Ferro

              
	 	Title: 
	Chairperson,
                Chief Executive Officer &  President

      

    

    
      
        
        

      

      
        6THIS
      NOTE AND THE COMMON SHARES ISSUABLE UPON CONVERSION OF THIS NOTE HAVE NOT BEEN
      REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED. THIS NOTE AND THE
      COMMON SHARES ISSUABLE UPON CONVERSION OF THIS NOTE MAY NOT BE SOLD, OFFERED
      FOR
      SALE, PLEDGED OR HYPOTHECATED IN THE ABSENCE OF AN EFFECTIVE REGISTRATION
      STATEMENT AS TO THIS NOTE UNDER SAID ACT OR AN OPINION OF COUNSEL REASONABLY
      SATISFACTORY TO THE TUBE MEDIA CORP. THAT SUCH REGISTRATION IS NOT
      REQUIRED.

    

     

    
      	Principal Amount
              $100,000.00	 	
              Issue
                Date: September 20,
                2006

            

    

         

     

    CONVERTIBLE
      NOTE

    

    FOR
      VALUE
      RECEIVED, THE TUBE MEDIA CORP., a Delaware corporation (hereinafter called
      "Borrower"), hereby promises to pay to Patrick LaPlatney located at 3000
      Devonshire Place, Atlanta, Georgia 30327 (the "Holder") or order, without
      demand, the sum of One Hundred Thousand Dollars ($100,000.00), with interest
      accruing thereon, on December 31, 2006 (the "Maturity Date"), if not retired
      sooner.

    

    The
      following terms shall apply to this Note:

    

    ARTICLE
      I

    

    GENERAL
      PROVISIONS

    

    1.1 Interest
      Rate.
      Interest shall accrue on this Note at the rate of 8% per annum based on 365
      day
      calendar year. Following the occurrence and during the continuance of an Event
      of Default, which, if susceptible to cure is not cured within ten (10) days,
      otherwise then from the first date of such occurrence, the annual interest
      rate
      on this Note shall (subject to Section 4.7) be twelve percent (12%) and
      calculated on a 365 day year.

    

    1.2 Maturity
      Date.
      Subject
      to the right of the Holder with respect to its conversion rights hereunder,
      all
      principal with interest accruing thereon is otherwise due on December 31, 2006
      (the "Maturity Date"); provided however, that should the Borrower close a
      subsequent financing transaction of either debt or equity in an amount exceeding
      $1,000,000 prior to the Maturity Date, then this Note shall become due and
      payable in full at such closing of the subsequent financing, inclusive of all
      interest

    

    1.3 Conversion
      Privileges.
      The
      Conversion Privileges set forth in Article II shall remain in full force and
      effect immediately from the occurrence of an Event of Default as defined in
      Article III hereof and until the Note is paid in full regardless of the
      subsequent cure of the Event of Default. The Note shall be payable in full
      on
      the Maturity Date, unless previously converted into Common Stock in accordance
      with Article II hereof.

    
      
        
        

      

      
        
        

        
          

        

      

      
        
        

      

    

    ARTICLE
      II

    

    CONVERSION
      RIGHTS

    

    The
      Holder shall have the right to convert the principal and any interest due under
      this Note into Shares of the Borrower's Common Stock, $.0001 par value per
      share
      (“Common Stock”) as set forth below.

    

    2.1. Conversion
      into the Borrower's Common Stock.

    

    (a) The
      Holder shall have the right from and after the date of the occurrence of an
      Event of Default and then at any time until this Note is fully paid, to convert
      any outstanding and unpaid principal portion of this Note, and accrued interest,
      at the election of the Holder (the date of giving of such notice of conversion
      being a "Conversion Date") into fully paid and nonassessable shares of Common
      Stock as such stock exists on the date of issuance of this Note, or any shares
      of capital stock of Borrower into which such Common Stock shall hereafter be
      changed or reclassified, at the conversion price as defined in Section 2.1(b)
      hereof (the "Conversion Price"), determined as provided herein. Upon delivery
      to
      the Borrower of a completed Notice of Conversion, a form of which is annexed
      hereto, Borrower shall issue and deliver to the Holder within three (3) business
      days after the Conversion Date (such third day being the “Delivery Date”) that
      number of shares of Common Stock for the portion of the Note converted in
      accordance with the foregoing. At the election of the Holder, the Borrower
      will
      deliver accrued but unpaid interest on the Note, if any, through the Conversion
      Date directly to the Holder on or before the Delivery Date (as defined in the
      Subscription Agreement). The number of shares of Common Stock to be issued
      upon
      each conversion of this Note shall be determined by dividing that portion of
      the
      principal of the Note and interest, if any, to be converted, by the Conversion
      Price.

    

    (b)  Subject
      to adjustment as provided in Section 2.1(c) hereof, the Conversion Price per
      share shall be $2.25 (“Conversion Price).

    

    (c) 
      The
      Conversion Price and number and kind of shares or other securities to be issued
      upon conversion determined pursuant to Section 2.1(a), shall be subject to
      adjustment from time to time upon the happening of certain events while this
      conversion right remains outstanding, as follows:

    

    A. Merger,
      Sale of Assets, etc. If the Borrower at any time shall consolidate with or
      merge
      into or sell or convey all or substantially all its assets to any other
      corporation, this Note, as to the unpaid principal portion thereof and accrued
      interest thereon, shall thereafter be deemed to evidence the right to purchase
      such number and kind of shares or other securities and property as would have
      been issuable or distributable on account of such consolidation, merger, sale
      or
      conveyance, upon or with respect to the securities subject to the conversion
      or
      purchase right immediately prior to such consolidation, merger, sale or
      conveyance. The foregoing provision shall similarly apply to successive
      transactions of a similar nature by any such successor or purchaser. Without
      limiting the generality of the foregoing, the anti-dilution provisions of this
      Section shall apply to such securities of such successor or purchaser after
      any
      such consolidation, merger, sale or conveyance.

    

    B. Reclassification,
      etc. If the Borrower at any time shall, by reclassification or otherwise, change
      the Common Stock into the same or a different number of securities of any class
      or classes that may be issued or outstanding, this Note, as to the unpaid
      principal portion thereof and accrued interest thereon, shall thereafter be
      deemed to evidence the right to purchase an adjusted number of such securities
      and kind of securities as would have been issuable as the result of such change
      with respect to the Common Stock immediately prior to such reclassification
      or
      other change.

    

    
      
        
        

      

      
        2

        
          

        

      

      
        
        

      

    

    C. Stock
      Splits, Combinations and Dividends. If the shares of Common Stock are subdivided
      or combined into a greater or smaller number of shares of Common Stock, or
      if a
      dividend is paid on the Common Stock in shares of Common Stock, the Conversion
      Price shall be proportionately reduced in case of subdivision of shares or
      stock
      dividend or proportionately increased in the case of combination of shares,
      in
      each such case by the ratio which the total number of shares of Common Stock
      outstanding immediately after such event bears to the total number of shares
      of
      Common Stock outstanding immediately prior to such event..

     

    (d) Whenever
      the Conversion Price is adjusted pursuant to Section 2.1(c) above, the Borrower
      shall promptly mail to the Holder a notice setting forth the Conversion Price
      after such adjustment and setting forth a statement of the facts requiring
      such
      adjustment.

    

    2.2 Method
      of Conversion.
      This
      Note may be converted by the Holder in whole or in part as described in Section
      2.1(a) hereof and the Subscription Agreement. Upon partial conversion of this
      Note, a new Note containing the same date and provisions of this Note shall,
      at
      the request of the Holder, be issued by the Borrower to the Holder for the
      principal balance of this Note and interest which shall not have been converted
      or paid.

    

    2.3 Maximum
      Conversion.
      The
      Holder shall not be entitled to convert on a Conversion Date that amount of
      the
      Note in connection with that number of shares of Common Stock which would be
      in
      excess of the sum of (i) the number of shares of Common Stock beneficially
      owned
      by the Holder and its affiliates on a Conversion Date, (ii) any Common Stock
      issuable in connection with the unconverted portion of the Note, and (iii)
      the
      number of shares of Common Stock issuable upon the conversion of the Note with
      respect to which the determination of this provision is being made on a
      Conversion Date, which would result in beneficial ownership by the Holder and
      its affiliates of more than 4.99% of the outstanding shares of Common Stock
      of
      the Borrower on such Conversion Date. For the purposes of the provision to
      the
      immediately preceding sentence, beneficial ownership shall be determined in
      accordance with Section 13(d) of the Securities Exchange Act of 1934, as
      amended, and Regulation 13d-3 thereunder. Subject to the foregoing, the Holder
      shall not be limited to aggregate conversions of only 4.99% and aggregate
      conversion by the Holder may exceed 4.99%. The Holder shall have the authority
      and obligation to determine whether the restriction contained in this Section
      2.3 will limit any conversion hereunder and to the extent that the Holder
      determines that the limitation contained in this Section applies, the
      determination of which portion of the Notes are convertible shall be the
      responsibility and obligation of the Holder. The Holder may waive the conversion
      limitation described in this Section 2.3, in whole or in part, upon and
      effective after 61 days prior written notice to the Borrower to increase such
      percentage to up to 9.99%. 

     

    ARTICLE
      III

    

    EVENT
      OF DEFAULT

    

    The
      occurrence of any of the following events of default ("Event of Default") shall,
      at the option of the Holder hereof, make all sums of principal and interest
      then
      remaining unpaid hereon and all other amounts payable hereunder immediately
      due
      and payable, upon demand, without presentment, or grace period, all of which
      hereby are expressly waived, except as set forth below:

    

    3.1 Failure
      to Pay Principal or Interest.
      The
      Borrower fails to pay any installment of principal, interest or other sum due
      under this Note when due and such failure continues for a period of ten (10)
      days after the due date. The ten (10) day period described in this Section
      3.1
      is the same ten (10) day period described in Section 1.1 hereof.

    

    
      
        
        

      

      
        3

        
          

        

      

      
        
        

      

    

    3.2 Breach
      of Covenant.
      The
      Borrower breaches any material covenant of this Note in any material respect
      and
      such breach, if subject to cure, continues for a period of ten (10) business
      days after written notice to the Borrower from the Holder.

    

    3.3 Breach
      of Representations and Warranties.
      Any
      material representation or warranty of the Borrower made herein, or in any
      agreement, statement or certificate given in writing pursuant hereto or in
      connection therewith shall be false or misleading in any material respect as
      of
      the date made and the Closing Date, and would otherwise have a material adverse
      effect on the Borrower.

    

    3.4 Receiver
      or Trustee.
      The
      Borrower shall make an assignment for the benefit of creditors, or apply for
      or
      consent to the appointment of a receiver or trustee for it or for a substantial
      part of its property or business; or such a receiver or trustee shall otherwise
      be appointed.

    

    3.5 Judgments.
      Any
      money judgment, writ or similar final process shall be entered or filed against
      Borrower or any of its property or other assets for more than $500,000, and
      shall remain unvacated, unbonded or unstayed for a period of forty-five (45)
      days.

    

    3.6 Bankruptcy.
      Bankruptcy, insolvency, reorganization or liquidation proceedings or other
      proceedings or relief under any bankruptcy law or any law, or the issuance
      of
      any notice in relation to such event, for the relief of debtors shall be
      instituted by or against the Borrower and if instituted against Borrower are
      not
      dismissed within 45 days of initiation.

    

    3.7  Delisting.
      Delisting of the Common Stock from any Principal Market; failure to comply
      with
      the requirements for continued listing on a Principal Market for a period of
      seven consecutive trading days; or notification from a Principal Market that
      the
      Borrower is not in compliance with the conditions for such continued listing
      on
      such Principal Market.

    

    3.8 Non-Payment.
      A
      default by the Borrower under any one or more obligations in an aggregate
      monetary amount in excess of $500,000 for more than twenty days after the due
      date, unless the Borrower is contesting the validity of such obligation in
      good
      faith.

    

    3.9 Stop
      Trade.
      An SEC
      or judicial stop trade order or Principal Market trading suspension that lasts
      for five or more consecutive trading days.

    

    3.10 Failure
      to Deliver Common Stock or Replacement Note.
      Borrower's failure to timely deliver Common Stock to the Holder pursuant to
      and
      in the form required by this Note or, if required, a replacement
      Note.

    

    3.11 Reservation
      Default.
      Failure
      by the Borrower to have reserved for issuance upon conversion of the Note the
      amount of Common stock as set forth in this Note.

    

    
      
        
        

      

      
        4

        
          

        

      

      
        
        

      

    

    ARTICLE
      IV

     

    HOLDER
      REPRESENTATIONS

    

    Holder
      hereby represents and warrants to and agrees with the Company only as to such
      Holder that:

    

    (a) Information
      on Company.
      The
      Holder has been furnished with or has had access at the EDGAR Website of the
      Commission to the Company's Form 10-KSB for the year ended December 31, 2005
      and
      all periodic reports filed with the Commission thereafter not later than five
      days before the Closing Date (hereinafter referred to as the "Reports").
      In
      addition, the Holder has received in writing from the Company such other
      information concerning its operations, financial condition and other matters
      as
      the Holder has requested in writing (such other information is collectively,
      the
      "Other
      Written Information"),
      and
      considered all factors the Holder deems material in deciding on the advisability
      of investing in the Securities. 

    

    (b) Information
      on Holder.
      The
      Holder is, and will be at the time of the conversion of the Notes, an
      "accredited investor", as such term is defined in Regulation D promulgated
      by
      the Commission under the 1933 Act, is experienced in investments and business
      matters, has made investments of a speculative nature and has purchased
      securities of United States publicly-owned companies in private placements
      in
      the past and, with its representatives, has such knowledge and experience in
      financial, tax and other business matters as to enable the Holder to utilize
      the
      information made available by the Company to evaluate the merits and risks
      of
      and to make an informed investment decision with respect to the proposed
      purchase, which represents a speculative investment. The Holder has the
      authority and is duly and legally qualified to purchase and own the Securities.
      The Holder is able to bear the risk of such investment for an indefinite period
      and to afford a complete loss thereof. The information set forth on the
      signature page hereto regarding the Holder is accurate.

    

    (c) Purchase
      of Notes.
      On the
      Closing Date, the Holder will purchase the Notes as principal for its own
      account for investment only and not with a view toward, or for resale in
      connection with, the public sale or any distribution thereof, but Holder does
      not agree to hold the Notes and Warrants for any minimum amount of
      time.

    

    (d) Compliance
      with Securities Act.
      The
      Holder understands and agrees that the Securities have not been registered
      under
      the 1933 Act or any applicable state securities laws, by reason of their
      issuance in a transaction that does not require registration under the 1933
      Act
      (based in part on the accuracy of the representations and warranties of Holder
      contained herein), and that such Securities must be held indefinitely unless
      a
      subsequent disposition is registered under the 1933 Act or any applicable state
      securities laws or is exempt from such registration. Notwithstanding anything
      to
      the contrary contained in this Agreement, such Holder may transfer (without
      restriction and without the need for an opinion of counsel) the Securities
      to
      its Affiliates (as defined below) provided that each such Affiliate is an
“accredited investor” under Regulation D and such Affiliate agrees to be bound
      by the terms and conditions of this Agreement. For the purposes of this
      Agreement, an “Affiliate”
of
      any
      person or entity means any other person or entity directly or indirectly
      controlling, controlled by or under direct or indirect common control with
      such
      person or entity. Affiliate when employed in connection with the Company
      includes each Subsidiary [as defined in Section 5(a)] of the Company. For
      purposes of this definition, “control”
means
      the power to direct the management and policies of such person or firm, directly
      or indirectly, whether through the ownership of voting securities, by contract
      or otherwise.

     

    (e) Shares
      Legend.
      The
      Shares shall bear the following or similar legend:

     

    
      
        
        

      

      
        5

        
          

        

      

      
        
        

      

    

    "THE
      SHARES REPRESENTED BY THIS CERTIFICATE HAVE NOT BEEN REGISTERED UNDER THE
      SECURITIES ACT OF 1933, AS AMENDED. THESE SHARES MAY NOT BE SOLD, OFFERED FOR
      SALE, PLEDGED OR HYPOTHECATED IN THE ABSENCE OF AN EFFECTIVE REGISTRATION
      STATEMENT UNDER SUCH SECURITIES ACT OR ANY APPLICABLE STATE SECURITIES LAW
      OR AN
      OPINION OF COUNSEL REASONABLY SATISFACTORY TO THE TUBE MEDIA CORP. THAT SUCH
      REGISTRATION IS NOT REQUIRED."

     

    

    (f) Note
      Legend.
      The
      Note shall bear the following legend:

     

    "THIS
      NOTE AND THE COMMON SHARES ISSUABLE UPON CONVERSION OF THIS NOTE HAVE NOT BEEN
      REGISTERED UNDER THE SECURITIES ACT OF 1933, AS AMENDED. THIS NOTE AND THE
      COMMON SHARES ISSUABLE UPON CONVERSION OF THIS NOTE MAY NOT BE SOLD, OFFERED
      FOR
      SALE, PLEDGED OR HYPOTHECATED IN THE ABSENCE OF AN EFFECTIVE REGISTRATION
      STATEMENT AS TO THIS NOTE UNDER SAID ACT OR AN OPINION OF COUNSEL REASONABLY
      SATISFACTORY TO THE TUBE MEDIA CORP. THAT SUCH REGISTRATION IS NOT
      REQUIRED."

     

    (g) Communication
      of Offer.
      The
      offer to sell the Securities was directly communicated to the Holder by the
      Company. At no time was the Holder presented with or solicited by any leaflet,
      newspaper or magazine article, radio or television advertisement, or any other
      form of general advertising or solicited or invited to attend a promotional
      meeting otherwise than in connection and concurrently with such communicated
      offer.

     

    (h) Authority;
      Enforceability.
      This
      Agreement and other agreements delivered together with this Agreement or in
      connection herewith have been duly authorized, executed and delivered by the
      Holder and are valid and binding agreements enforceable in accordance with
      their
      terms, subject to bankruptcy, insolvency, fraudulent transfer, reorganization,
      moratorium and similar laws of general applicability relating to or affecting
      creditors’ rights generally and to general principles of equity; and Holder has
      full corporate power and authority necessary to enter into this Agreement and
      such other agreements and to perform its obligations hereunder and under all
      other agreements entered into by the Holder relating hereto.

    

    (i) No
      Governmental Review.
      Holder
      understands that no United States federal or state agency or any other
      governmental or state agency has passed on or made recommendations or
      endorsement of the Securities or the suitability of the investment in the
      Securities nor have such authorities passed upon or endorsed the merits of
      the
      offering of the Securities.

    

    (j) Correctness
      of Representations.
      Holder
      represents as that the foregoing representations and warranties are true and
      correct as of the date hereof and, unless a Holder otherwise notifies the
      Company prior to the Closing Date shall be true and correct as of the Closing
      Date.

    

    (k) Survival.
      The
      foregoing representations and warranties shall survive the Closing Date until
      three years after the Closing Date.

    

    
      
        
        

      

      
        6

        
          

        

      

      
        
        

      

      ARTICLE
        V

       

      MISCELLANEOUS

    

    

    5.1 Failure
      or Indulgence Not Waiver.
      No
      failure or delay on the part of Holder hereof in the exercise of any power,
      right or privilege hereunder shall operate as a waiver thereof, nor shall any
      single or partial exercise of any such power, right or privilege preclude other
      or further exercise thereof or of any other right, power or privilege. All
      rights and remedies existing hereunder are cumulative to, and not exclusive
      of,
      any rights or remedies otherwise available.

    

    5.2 Notices.
      All
      notices, demands, requests, consents, approvals, and other communications
      required or permitted hereunder shall be in writing and, unless otherwise
      specified herein, shall be (i) personally served, (ii) deposited in the mail,
      registered or certified, return receipt requested, postage prepaid, (iii)
      delivered by reputable air courier service with charges prepaid, or (iv)
      transmitted by hand delivery, telegram, or facsimile, addressed as set forth
      below or to such other address as such party shall have specified most recently
      by written notice. Any notice or other communication required or permitted
      to be
      given hereunder shall be deemed effective (a) upon hand delivery or delivery
      by
      facsimile, with accurate confirmation generated by the transmitting facsimile
      machine, at the address or number designated below (if delivered on a business
      day during normal business hours where such notice is to be received), or the
      first business day following such delivery (if delivered other than on a
      business day during normal business hours where such notice is to be received)
      or (b) on the second business day following the date of mailing by express
      courier service, fully prepaid, addressed to such address, or upon actual
      receipt of such mailing, whichever shall first occur. The addresses for such
      communications shall be: (i) if to the Borrower to: The Tube Media Corp., 1451
      West Cypress Creek Road, Ft. Lauderdale, FL 33309, Attn: David Levy, telecopier:
      954-714-8500, with a copy by telecopier only to: Blank Rome LLP, 1200 N. Federal
      Highway, Suite 417, Boca Raton, FL 33432, Attn: Bruce C. Rosetto, Esq.,
      telecopier: (561) 417-8186, and (ii) if to the Holder, to the name, address
      and
      telecopy number set forth on the front page of this Note.

    

    5.3 Amendment
      Provision.
      The
      term "Note" and all reference thereto, as used throughout this instrument,
      shall
      mean this instrument as originally executed, or if later amended or
      supplemented, then as so amended or supplemented.

    

    5.4 Assignability.
      This
      Note shall be binding upon the Borrower and its successors and assigns, and
      shall inure to the benefit of the Holder and its successors and
      assigns.

    

    5.5 Cost
      of Collection.
      If
      default is made in the payment of this Note, Borrower shall pay the Holder
      hereof reasonable costs of collection, including reasonable attorneys'
      fees.

    

    5.6 Governing
      Law.
      This
      Note shall be governed by and construed in accordance with the laws of the
      State
      of Florida. Any action brought by either party against the other concerning
      the
      transactions contemplated by this Agreement shall be brought only in the state
      courts of Florida or in the federal courts located in the state of Florida.
      Both
      parties and the individual signing this Agreement on behalf of the Borrower
      agree to submit to the jurisdiction of such courts. The prevailing party shall
      be entitled to recover from the other party its reasonable attorney's fees
      and
      costs.

    

    5.7 Maximum
      Payments.
      Nothing
      contained herein shall be deemed to establish or require the payment of a rate
      of interest or other charges in excess of the maximum permitted by applicable
      law. In the event that the rate of interest required to be paid or other charges
      hereunder exceed the maximum permitted by such law, any payments in excess
      of
      such maximum shall be credited against amounts owed by the Borrower to the
      Holder and thus refunded to the Borrower.

    

    
      
        
        

      

      
        7

        
          

        

      

      
        
        

      

    

    5.8 Shareholder
      Status.
      The
      Holder shall not have rights as a shareholder of the Borrower with respect
      to
      unconverted portions of this Note. However, the Holder will have all the rights
      of a shareholder of the Borrower with respect to the shares of Common Stock
      to
      be received by Holder after delivery by the Holder of a Conversion Notice to
      the
      Borrower.

    

    

    [THIS
      SPACE INTENTIONALLY LEFT BLANK]

    

    

     

     

     

     

     

     

     

    
 

    
      
        
        

      

      
        8

        
          

        

      

      
        
        

      

    

    IN
      WITNESS WHEREOF,
      Borrower has caused this Note to be signed in its name by an authorized officer
      as of the 20th day of September, 2006.

     

     

    
      	 	 	 
	 	THE
              TUBE MEDIA
              CORP.
	 
 	 
 	 
 
	 	By:  	/s/ Celestine
              F. Spoden
	 	
              
Name:
              Celestine F. Spoden
	 	Title:
              Chief Financial Officer

WITNESS:

    
 

    /s/
      Deborah Ely

    Deborah
      Ely

     

     

    
      
        
        

      

      
        9

        
          

        

      

      
        
        

      

    

    NOTICE
      OF CONVERSION

     

    

    (To
      be
      executed by the Registered Holder in order to convert the Note)

    

    

    The
      undersigned hereby elects to convert $_________ of the principal and $_________
      of the interest due on the Note issued by The Tube Media Corp. on September
      20,
      2006 into Shares of Common Stock of The Tube Media Corp. (the "Borrower")
      according to the conditions set forth in such Note, as of the date written
      below.

    

    

    
      	Date
              of
              Conversion: 	 	 
	 	 	 
	Conversion
              Price: 	 	 
	 	 	 
	Shares
              To Be Delivered:	 	 
	 	 	 
	Signature:
	 	 
	 	 	 
	Print
              Name:	 	 
	 	 	 
	Address:	 	 
	 	 	 

    

     

    
 

    
      
        
        

      

      
        10

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