Cancer arising at an ileostomy site represents a rare late complication of total colectomy performed for ulcerative colitis. There are no more than 36 published cases in the literature. We describe a case of adenocarcinoma of the mucocutaneous junction at the ileostomy site, occurring 31 years after total colectomy for ulcerative colitis. Wide excision of the moderately differentiated adenocarcinoma was performed with refashioning of the ileostomy. Polypoid adenomas or adenocarcinomas of the ileostomy after colectomy performed for non-neoplastic conditions are extremely uncommon. Biopsies of polypoid lesions at the stoma site are recommended.