Neoplastic lesions of the ampulla of Vater are a rare cause of acute and recurrent pancreatitis. We have recently had the privilege of studying a 58-year-old woman who presented with recurrent pancreatitis. At endoscopic retrograde pancreatography, a mucosal abnormality of the ampulla of Vater was noted and documented to be a villous adenoma. It was resected surgically and the patient has been symptom free. This report documents the ninth patient in the English literature with pancreatitis secondary to a benign neoplasm of the ampulla of Vater. Six of the nine patients have been male. They have ranged in age from 42 to 68 years. Approximately 30% of villous adenomas of the duodenum contain foci of invasive or in situ carcinoma. Villous adenomas of the ampulla of Vater require aggressive therapy. Further, it has been noted that a number of patients with small intestinal neoplasms have concomitant large-bowel polyps.