A 41-year-old man presented with a 6-mo history of changed defecation and rectal bleeding. A 3-cm polypoid tumor of the lower rectum was found at rectosigmoidos- copy, which proved to be a leiomyosarcoma upon biopsy. Dissemination studies did not show any metastases. He was underwent to an abdomino-perineal resection (APR). Histopathology of the specimen showed a melanoma (S-100 stain positive). Two years after the resection, me- tastases in the abdomen and right lung were found. He died one and half years later. Primary anorectal melano- ma is a rare and very aggressive disorder. According to current data, one should always perform a S-100 stain when anorectal sarcoma is suspected. A positive S-100 stain suggests the tumour to be most likely a melanoma. Subsequently, thorough dissemination studies need to be performed. Depending on the outcome of the dissemina- tion studies, a surgical resection has to be performed. Nowadays, a sphincter-saving local excision combined with adjuvant loco-regional radiotherapy should be pre- ferred in case of small tumors. The same loco-regional control is achieved with less “loss of function” compared to non-sphincter saving surgery. Only in the case of large and obstructing tumors an abdomino-perineal resection is the treatment of choice. A 41-year-old man presented with a 6-mo history of altered defecation and rectal bleeding. A 3-cm polypoid tumor of the lower rectum was found at rectosigmoidos-copy, which proved to be a leiomyosarcoma upon biopsy. Dissemination studies did Not show any metastases. He was underwent to an abdomino-perineal resection (APR). Histopathology of the specimen showed a melanoma (S-100 stain positive). Two years after the resection, me- tastases in the abdomen and right lung were found He died one and half years later. Primary anorectal melano- ma is a rare and very aggressive disorder. According to the current data, one should always perform a S-100 stain when anorectal sarcoma is suspected. A positive S-100 stain suggests the Now on the outcome of the dissemina- tion studies, a surgical resection has to be performed. Nowadays, a sphincter-saving local excision combined with adjuvant lo co-regional radiotherapy should be pre- ferred in case of small tumors. The same loco-regional control is achieved with less “loss of function ” compared to non-sphincter saving surgery. Only in the case of large and obstructing tumors an abdomino-perineal resection is the treatment of choice.