The BRCA1-associated protein1 (BAP1) syndrome is a tumor predisposition syndrome dependent on the presence of germline pathogenic variants at the level of the tumor suppressor gene BAP1 that predisposes to the development of various types of tumors including uveal melanoma, mesothelioma, cutaneous melanoma, and RCC [33]. The gene discussed is BAP1; the disease is renal cell carcinoma.