Uveal and conjunctival melanomas comprise approximately 5% of all melanomas. The incidence of uveal melanoma in the United States is similar to that in European countries. The overall mean incidence of uveal melanoma in the United States is 5.2 per million per year with a higher rate in males (6.0 per million per year) as compared to females (4.5 per million per year). Uveal melanoma is more common in the older age group, with a progressively rising age-specific incidence that peaks at the age of 70 years. The age-adjusted annual incidence of uveal melanoma in the United States has remained stable for the last 50 years. Known causes of uveal melanoma include congenital ocular melanocytosis, melanocytoma, and the BAP1 cancer predisposition syndrome, which is associated with cutaneous atypical nevi and melanoma and specific forms of SMN (second malignant neoplasms) such as malignant mesothelioma, meningioma, and lung adenocarcinoma. In the large majority of cases, the etiology is unknown, although ocular melanomas are most common in fair individuals with light-colored eyes.