MAJOR SHAREHOLDER ANNOUNCEMENT

Copenhagen, July 6, 2018 – Orphazyme A/S (the “Company”), a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, hereby announces the receipt of notification pursuant to Section 38 of the Danish Capital Markets Act from Danske Bank A/S that its indirect shareholding in the Company is 1,029,081 shares corresponding to 3.28% of the share capital in the Company and that Danske Bank A/S indirectly has control over 5.16% of the voting rights in the Company.