Adenomyoma is a nonneoplastic lesion that can be found anywhere in the gastrointestinal tract, but it's rarely found in the ampulla of Vater. To the best of our knowledge, it is a benign lesion, but most cases are misdiagnosed as carcinoma or adenoma by a preoperative endoscopic or radiologic procedure, and this leads to unnecessarily extensive surgical resection. We report here on a case of ampulla of Vater adenomyoma that resulted in biliary and pancreatic duct dilatation. The tumor was diagnosed by endoscopic papillectomy. (Korean J Gastrointest Endosc 2010;40:391-395)