Primary adenocarcinoma in a permanent ileostomy carries a poor prognosis from other gastrointestinal malignancies. Surveillance and identification of patient at risk for ileostomy malignancies is a challenging problem. There are not reliable biological markers. The clinical evaluation, suspicion of the disease, common presenting symptoms including difficulty fitting the stomal appliance, bowel obstruction, and a friable mass should be considered as part of the evaluation and screening in a long standing terminal ileostomy. Biopsy of newly developed lesions in the periostomal area is recommended for diagnosis and treatment. This is a case of a primary adenocarcinoma in an ileostomy forty years after total colectomy for ulcerative colitis.