Germline pathogenic mutations in the tumor suppressor gene BRCA1-associated protein-1 (BAP1) lead to BAP1 tumor predisposition syndrome (BAP1-TPDS) [6], which is characterized by high susceptibility to several tumor types, mainly melanoma (especially uveal), mesothelioma, renal cell carcinoma, and basal cell carcinoma (Table 2). The gene discussed is BAP1; the disease is melanoma.