FH and renal cell carcinoma: <h4>Introduction</h4>Fumarate Hydratase-deficient-renal cell carcinoma (FH-dRCC) is an uncommon and extremely aggressive variant of renal cell carcinoma (RCC) resulting from inactivating mutations in the tumor suppressor gene, fumarate hydratase (FH).<h4>Case presentation</h4>We report a case of a young male who presented with the complaint of painless hematuria.