The suspicion of shared genetic susceptibility to the development of second primary cancer among patients with UM has been emphasized by the recent identification of the BRCA1-Associated Protein 1 (BAP1) tumor predisposition syndrome presenting with a pathogenic germline variation in the BAP1 tumor suppressor gene, which is located on chromosome 3, and an increased incidence of uveal melanoma, renal cell carcinoma, mesothelioma, and cutaneous melanoma in the affected family members [6,7]. Here, BAP1 is linked to BAP1-related tumor predisposition syndrome.