Namely, several studies have demonstrated that carriers of BRCA1-associated protein 1 (BAP1) germline mutations can develop a variety of tumor types, including mesothelioma, uveal and cutaneous melanoma, and renal cell carcinoma; less frequently, breast cancer, different types of skin carcinomas, and other neoplasms have been observed [7]. This evidence concerns the gene BAP1 and renal cell carcinoma.