Thereafter, we and others confirmed that germline BAP1 mutations are a common heritable factor that predispose to MM, UM, cutaneous melanoma (CM), cholangiocarcinoma, renal cell carcinoma (RCC), and basal cell carcinoma (BCC) [4–6], and to benign atypical melanocytic lesions known as MBAITs [7, 8], and likely to several other malignancies including brain, breast, lung cancer, and sarcomas [9],—recently grouped together into the “BAP1 cancer syndrome” [7]. Here, BAP1 is linked to hereditary clear cell renal cell carcinoma.