OBJECTIVES: To describe the presentation, management, and clinical outcome of patients with genitourinary melanoma. METHODS: We identified 14 patients with genitourinary melanoma treated at Memorial Sloan-Kettering Cancer Center, New York and Tripler Army Medical Center, Honolulu, Hawaii. The presentation, surgical treatment, disease progression, and outcome of these patients were reviewed. Survival was analyzed, using the Kaplan-Meier product limit method. RESULTS: The presentation and management of patients with genitourinary melanoma were varied. Overall, the prognosis was poor, with a median survival of 43 months, and only 3 patients were alive, without disease, at last follow-up. Our findings confirm a poor prognosis in patients with this rare disease. CONCLUSIONS: Genitourinary melanoma is a rare form of the disease with an unfavorable clinical outcome. Less than one third of patients survive long term, although patients with scrotal melanomas may have a better prognosis.