Primary adenocarcinoma rarely develops at the site of an ileostomy performed for ulcerative colitis (UC), familial adenomatous polyposis (FAP), or Crohn's disease. We describe a case of ileostomy cancer found 14 years after proctocolectomy for FAP with cancer of both the sigmoid colon and rectum. Resection of the ileal mucosa around the stoma was performed three times. To our knowledge, only 35 other such cases have ever been reported. Our review of these cases indicates that routine examination of the stoma by a physician, or even by the patient, may lead to earlier detection of this rare complication, and a better chance of cure through minimal surgery.