Pancreaticobiliary maljunction (PBM) complicated with complete pancreas divisum

A rare pediatric case of pancreaticobiliary maljunction (PBM) with pancreas divisum is reported. The case showed extremely rare arrangement of the biliary tree and pancreatic duct; the common channel usually seen in PBM did not exist and the common bile duct was connected to the dorsal pancreatic duct via a dilated intermediary duct, in which a large protein plug had formed. The common bile duct was not dilated at all. All of these anomalies may have been caused by abnormal development of the ventral and dorsal pancreatic ducts at an early gestational age.