The BRCA1-associated protein 1 (BAP1) tumor predisposition syndrome (BAP1-TPDS) is an autosomal dominant cancer syndrome in which uveal melanoma has been the most frequently observed malignancy. Other core cancers associated with BAP1-TPDS include malignant mesothelioma, cutaneous melanoma, and renal cell carcinoma. The lifetime estimated risk of cancer for patients affected by this syndrome is as high as 85%. The estimated point prevalence of uveal melanoma in BAP1-TPDS is 2.8%. Patients with BAP1-TPDS who are affected by uveal melanoma present at younger age with larger tumor basal diameter and more frequent ciliary body involvement than patients diagnosed with uveal melanoma in the general population. Patients with BAP1-TPDS are likely to have a high-risk uveal melanoma gene expression profile, with increased risk of metastasis and uveal melanoma-related death. Patients with BAP1-TPDS should be offered screening and lifelong surveillance for uveal melanoma and other BAP1-associated cancers with a multidisciplinary team.