Annular pancreas is a rare congenital anomaly that rarely occurs in parallel with malignancy. We herein report a case of annular pancreas with carcinoma of the papilla of Vater. A 76-year-old woman presented with abdominal pain and was referred to us after gastroduodenal endoscopy showed a tumor of the papilla. Preoperative computed tomography confirmed the presence of an ampullary tumor. During surgery, we found an anomaly consisting of a ring-like band of pancreatic tissue encircling the second part of the duodenum. Transduodenal papillectomy with preservation of the annular pancreas was subsequently performed. The patient was discharged without any postoperative morbidity.

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