Tazemetostat is an epigenetic therapy and first-in-class EZH2 inhibitor that gained FDA accelerated approval in 2020 for patients greater than or equal to 16 years old with SMARCB1/INI1-deficient metastatic or locally advanced epithelioid sarcoma (ES) and adults with EZH2-mutated relapsed or refractory follicular lymphoma (FL). This evidence concerns the gene SMARCB1 and epithelioid sarcoma.