The focus on the genetic susceptibility of cancer among patients with uveal melanoma has increased with the recognition of BRCA1-Associated Protein 1 (BAP1) tumor predisposition syndrome presenting with an increased incidence of uveal melanoma, renal cell carcinoma, mesothelioma, and cutaneous melanoma in the affected family members. This evidence concerns the gene BAP1 and hereditary clear cell renal cell carcinoma.