Lymphomas are the third most common cancer in children and Burkitt lymphoma (BL) accounts for about 40% of them. The rectum is extremely seldom involved in BL: 91 children were treated at our institution for BL between 1987 and June 2006, but none of them had involved the rectum. We report here on a 9-year-old boy who presented with bowel bleeding and subocclusion, which proved due to a BL of the rectum. The role of surgery for this tumor is limited nowadays, except for surgical emergencies, biopsies, and second-look surgery in selected cases.