OBJECTIVE: To study the prevalence, clinical presentation, treatment, and follow-up of ocular (dermal) melanocytosis (ODM) and its association with choroidal melanoma (CM) in Asian Indian patients. METHODS: This was a retrospective case series of patients with ODM conducted in a quaternary eye care center. RESULTS: Of the total 1.48 million patients during the study period, we identified 213 patients with ODM with a prevalence rate of 0.014%. Median age at presentation was 13 years (range 0-76 years). Unilateral presentation of ODM was noted in 87% cases. ODM involved the sclera in 100% cases, the iris in 23%, the choroid in 20%, the eyelid in 29%, and V1 and V2 dermatomes in 53% cases. Nineteen patients (9%) were found to have CM, with 1.3 times increased risk of CM compared to the Caucasian population. The most common tumor location was in the inferior quadrant (n = 8, 44%). The mean tumor thickness was 8.3 ± 2.8 mm with a mean basal diameter of 14.0 ± 4.3 mm. Primary treatment of CM included plaque brachytherapy (n = 6), enucleation (n = 9), and few patients refused for treatment (n = 3). During a mean follow-up period of 21 months, one patient died because of lung metastasis. CONCLUSION: Although rare, choroidal melanoma does occur in the presence of ODM with an increased risk in Asian Indians compared to the Caucasian population. Patients with ODM should be examined periodically for early detection of CM.