A man in his 80's was admitted complaining of epigastralgia, and acute pancreatitis was diagnosed. Abdominal CT and MRI showed enlargement of the pancreatic head encircling the descending part of the duodenum and a duodenal diverticulum. Endoscopic retrograde cholangio-pancreatography (ERCP) revealed annular pancreas and high confluence of pancreaticobiliary ducts. Annular pancreas had been reported to have associated with a broad spectrum of pancreatic anomalies, few cases of annular pancreas coexisting with high confluence of pancreaticobiliary ducts, or pancreaticobiliary maljunction have been reported. Combination of both anomalies was interesting from the view point of embryology. We report a rare case of annular pancreas with high confluence of pancreaticobiliary ducts.