Anal melanoma is rare and aggressive malignancy. Patients commonly present with advanced, even metastatic disease. Unlike cutaneous melanoma, anal melanoma has no known risk factors. Surgical excision remains the cornerstone of therapy. There are no long-term survivors of stage II or III disease; therefore, early diagnosis and treatment remain crucial. There are no trials definitively proving abdominal perineal resection (APR) or wide local excision (WLE) to yield superior long-term survival. APR may offer a higher rate of local control, whereas WLE offers a much less morbid operation. Adjuvant chemotherapy, interferon, and radiation may offer some benefit.