BAP1 (BRCA1-associated protein 1) with its gene product acting as a deubiquitinating enzyme, is a critical tumor suppressor gene that is mutated in various human cancers, including metastatic uveal melanoma, pleural mesothelioma, and renal cell carcinoma [1,2,3,4]. The gene discussed is BAP1; the disease is hereditary clear cell renal cell carcinoma.