Objectives: The main objective of this review is to critically evaluate the present evidence regarding a relationship between ocular melanoma (OM) and cutaneous melanoma (CM). Melanomas are malignant neoplasms that develop from dendritic melanocytes that are found in the skin, eye, mucosal epithelia, and leptomeninges. While the skin is the most common site of melanoma development, these neoplasms can occur in any tissue that contains melanocytes. We review the incidence and characteristics of OM, evaluate the existent data regarding its potential relationship to CM, and provide some guidance for the dermatologist regarding the evaluation and diagnosis of this ocular tumor. Data Sources: A retrospective review of the literature. Study Selection: Studies included those relevant to disease pathogenesis and incidence, cohort studies (ie, studies evaluating the incidence of CM in patients with OM), and pertinent investigations from the dermatologic literature (ie, cases of atypical nevus syndromes). The referenced study designs and methodologies varied. Data Extraction and Synthesis: Data were extracted by 2 independent reviewers, and the main results are presented in a qualitative, descriptive manner. Conclusion: Evidence for comorbid OM and CM exists in patients with strong phenotypic expression of atypical nevi and conjunctival melanoma, although CDKN2A mutations have not been documented in patients with OM.