Pancreatic duct stones are commonly associated with recurrent pancreatitis. They are believed to develop as a result of the calcification of an intraductal protein plug. A choledochal cyst is a relatively rare anomaly usually presenting with abdominal pain, jaundice and palpable mass. APBDU (anomalous pancreaticobiliary ductal union) is frequently associated with various pancreatobiliary diseases, including choledochal cyst, biliary tumor, pancreatitis and pancreas divisum. We report a 48-year-old woman who presented with right upper quadrant pain with a pancreatic duct stone, a choledochal cyst and APBDU. She underwent endoscopic pancreatic sphincterotomy, a surgical choledochal cyst excision and Roux-en-Y choledochojejunostomy. (Korean J Gastrointest Endosc 2006; 33:130134)