Orphazyme A/S : MAJOR SHAREHOLDER ANNOUNCEMENT

Copenhagen, Denmark, January 18, 2021 - Orphazyme A/S (ORPHA.CO; ORPH) ('the Company'), a late-stage biopharmaceutical company pioneering the Heat-Shock Protein response for the treatment of neurodegenerative orphan diseases, hereby announces the receipt of notification pursuant to Section 38 of the Danish Capital Markets Act from Danske Bank A/S, that as of January 15, 2021, Danske Bank A/S holds a total of 34,697,703 shares in the Company, corresponding to 3.49% of the Company's share capital and that Danske Bank A/S as of January 15, 2021 controls 4.95% of the voting rights in the Company.

Danske Bank A/S' shareholding consists of a 2.24% indirect and 1.25% direct ownership through Danica Pension Livsforsikringsaktieselskab, Danica Pension Försäkringsaktiebolag, Investeringsforeningen Danske Invest and Danske Invest SICAV. Danske Bank A/S' control of voting rights in the Company consists of a 3.70% indirect and 1.25% direct control through Danica Pension Livsforsikringsaktieselskab, Danica Pension Försäkringsaktiebolag, Investeringsforeningen Danske Invest and Danske Invest SICAV.