To describe the clinical features, natural course, management and histopathologic features of iris melanocytoma. Single-center retrospective case series. Forty-seven consecutive patients (47 eyes) with iris melanocytoma. INTERVENTION PROCEDURE: Data regarding patient and tumor features were analyzed for their impact on the main outcome measures using univariate and multivariate regression models. Kaplan-Meier estimates were used to analyze the main outcomes as a function of time. Increased intraocular pressure (IOP), tumor seeding, and tumor growth. Associated findings at initial presentation included iris stromal seeds in 20 patients (43%), and anterior chamber angle seeds in 12 (26%). Intrinsic vascularization and sector cataract were not seen in any eyes. The management at presentation included observation in 39 patients (83%), tumor removal by sector iridectomy/iridocyclectomy in 7 (15%), and enucleation for blind painful eye with secondary increased IOP in 1 (2%). The diagnosis was confirmed by histopathologic examination in 11 patients (23%). The mean follow-up was 58 months. Using Kaplan-Meier estimates, clinical evidence of growth was observed in 23% at 5 years, 48% at 10 years, and 74% at 15 years. New tumor seeds developed in 34% at 5 years, 63% at 10 years, and 75% at 15 years. Increased IOP was observed in 11% at 5 years, 11% at 10 years, and 55% at 15 years. Iris melanocytoma represented only 3% of all iris nevi. Related iris stromal and anterior chamber angle seeds were common, and secondary glaucoma occurred in 11% at 5 years. Growth was observed in 23% at 5 years but no malignant transformation was found.