Restorative proctocolectomy with ileal pouch-anal anastomosis has been accepted as the surgical treatment of choice for most patients with ulcerative colitis. The occurrence of adenocarcinoma arising near or into the ileal pouch is rare. Only 19 such cases have been reported so far. The authors report a case of a 67-year old male who developed an adenocarcinoma in the small rectal stump 12 years after a restorative proctocolectomy with double stapled ileal pouch-low rectal anastomosis for ulcerative colitis unresponsive to medical treatment. They, after a literature review, examine same steps of the procedure and emphasize the importance of regular and prolonged follow-up for all patients having restorative proctocolectomy for ulcerative colitis.