Folliculin (FLCN) is a tumor suppressor gene that is deficient in Birt-Hogg-Dube syndrome (BHD), a disorder that features renal carcinoma of multiple histological types including hybrid oncocytic RCC, chromophobe RCC, oncocytoma, multiple and bilateral clear cell RCC (Verine et al., 2010). This evidence concerns the gene FLCN and Birt-Hogg-Dubé syndrome.