Melanomas affecting different components of the uvea occur with differing frequencies and clinical presentations. Uveal melanoma is diagnosed via funduscopic exam and ancillary tests. These lesions may present with visual findings or incidental findings on physical exam. Metastasis occurs in approximately half of all patients with primary uveal melanoma. The liver is the most common site of metastasis. Enucleation was at one time considered the definitive local treatment for primary uveal melanoma, but has been largely replaced by other therapeutic procedures that aim to prevent metastasis while preserving vision. Unfortunately, metastasis of uveal melanoma almost always proves to be fatal. The current treatment of metastatic uveal melanoma is limited by the intrinsic resistance of uveal melanoma to conventional systemic therapies. Advancements in molecular biology have resulted in the identification of a number of promising prognostic and therapeutic targets. Early detection and therapy are important factors in disease survival. It is imperative that the treating physician be familiar with the clinical features of uveal melanoma and distinguish it from mimickers in order to ensure effective and timely treatment.