The germline BAP1 mutation is associated with an increased risk of UM [10, 11, 12, 13], mesothelioma [11, 13, 14], cutaneous melanoma [11, 13], meningioma [12], RCC [13, 15] and MBAITs [11] (melanocytic BAP1-mutated atypical intradermal tumors) and is known as BAP1 hereditary cancer predisposition syndrome [13]. Here, BAP1 is linked to renal cell carcinoma.