The principal autosomal dominant syndromes account for most hereditary RCC: von Hippel–Lindau (VHL), Hereditary Leiomyomatosis and RCC (HLRCC), Hereditary Papillary RCC (HPRC), Birt–Hogg–Dubé (BHD), and BAP1 tumor predisposition syndrome (BAP1-TPDS). This evidence concerns the gene BAP1 and BAP1-related tumor predisposition syndrome.