Abstract:Pancreaticobiliary maljunction (PBM) is a congenital anatomical anomaly in which the pancreatic duct and bile duct join outside the duodenal wall to form an abnormally long common channel. This anomaly disrupts the normal function of the sphincter of Oddi, leading to bidirectional reflux of pancreatic juice and bile and subsequently causing various hepatobiliary and pancreatic diseases. The early symptoms of PBM are insidious and nonspecific; however, delayed diagnosis and intervention significantly increase the risk of poor outcomes and seriously threaten the lives and health of affected children. Therefore, this article systematically reviews advances in the pathophysiological mechanisms, clinical classification criteria, diagnostic techniques, and treatment strategies of PBM to provide a reference for basic research and standardized clinical diagnosis and treatment.