Adenomatous polyps lead directly to carcinoma of the colon in patients with one of the familial syndromes of intestinal polyposis. Elective colectomy is prophylactic and life-saving. A subgroup of patients will develop periampullary carcinoma, again arising from the presence of adenomatous polyps. Such a case is presented. These polyps should be surgically excised to ensure adequate pathologic examination. Yearly upper endoscopic examination is an essential element of management. Either the presence of carcinoma in situ or the recurrence of these polyps following excision is confirmation of behavior with a high propensity for the development of invasive cancer and requires aggressive treatment.