This report described a patient who presented with biliary tract obstruction caused by ampullary hamartoma. Duodenoscopy showed a large mass on the ampulla of Vater. Endoscopic biopsy revealed no evidence of malignancy. Frozen section biopsies of the tumor performed during operation disclosed hamartoma without evidence of malignancy. Therefore, simple local excision and sphincteroplasly were performed. The serial histological sections of the resected specimen confirmed a hamartoma of the ampulla of Vater. Hamartomas are unusual tumors in the duodenum; we, therefore, present this very rare case.