The specific genetic mechanisms responsible for the malignant behavior of uveal melanoma are not known. Unlike cutaneous melanoma, epidemiologic studies have not demonstrated a definitive germline form of uveal melanoma, though familial melanoma and racial predilections occur. Molecular cytogenetic characterization of uveal melanoma suggests that somatic deletions of chromosome 3 are associated with a worse prognosis. Microarray technology has been used to characterize uveal melanoma gene expression and may provide tests useful for determining prognosis. As an improved understanding of the cellular mechanisms used by uveal melanoma is gained, new opportunities to adapt or design therapeutic approaches may emerge.