Pancreaticobiliary maljunction (PBM) is a rare congenital malformation defined as a union of the pancreatic duct and the bile duct outside the duodenal wall. PBM has become eye-catching due to its frequent association with the occurrence of a variety of biliary and pancreatic diseases. Here, the authors address the progress in terms of classification, pathological and pathophysiological process, as well as early diagnosis and treatment of PBM.