Anorectal melanoma (ARM) is an extremely rare and aggressive malignancy. There is no standard therapeutic management, mainly due to scarcity of data and randomized control trials regarding the optimal surgical strategy. We present the case of a male patient aged 65 years in our hospital, presenting perineal pain and rectal bleeding. Patient’s performance status (PS) was good. Hematological analysis revealed normocytic normochromic anemia. In the rectoscopy, an anal canal tumor formation was identified. The diagnosis of ulcerated melanoma was confirmed at the histopathological examination. The pelvic MRI detected a circumferential parietal thickening, posteriorly accentuated at the level of the anal canal, about 4.4 cm long, situated 2 cm from the external anal orifice, causing a subtotal lumen stenosis. The patient underwent abdominoperineal resection (APR). Postoperative course was favorable and the patient was discharged on the 13th postoperative day, with no complications. A multidisciplinary cancer care team will prove to be of substantial value, because it may develop a personalized treatment plan for this unusual tumor. Although the therapeutic approach is still controversial, surgery is considered the mainstay therapy.