Angiomyolipoma of the colon is very rare. Only three cases have been reported in the literature. Here we report the case of a 54-year-old male, who presented with a progressive abdominal cramping pain. We performed left hemicolectomy under the impression of a cancerous mass over the splenic flexure of the colon. Histology revealed an angiomyolipoma of the colon. In addition, we review the literature.