Germline deleterious variants in the BAP1 gene are associated with the BAP1 tumor predisposition syndrome, an autosomal dominant condition characterized by an increased risk for atypical Spitz tumors and other types of cancer, such as uveal melanoma, malignant mesothelioma, cutaneous melanoma, clear cell renal cell carcinoma, and basal cell carcinoma [107]. Here, BAP1 is linked to BAP1-related tumor predisposition syndrome.