ObjectiveTo discuss the diagnosis and endoscopic therapy of pancreaticobiliary maljunction by multidisciplinary team (MDT).MethodThe preoperative MDT discussion and the diagnosis and treatment process of patient with pancreaticobiliary maljunction in the Fifth People’s Hospital of Zunyi in 2019 were summarized.ResultsThe patient was admitted for “upper abdominal pain approximately 10 h”. The obvious extramural confluence of the pancreaticobiliary tract was observed and the length of common channel was approximately 1.8 cm. But the junction of the pancreaticobiliary tract was obviously controlled by the sphincter of Oddi, and the amylase value of the bile was higher than that of the serum. After the MDT discussion, there were still doubts about the diagnosis of pancreaticobiliary maljunction or high confluence of pancreaticobiliary ducts. After the left hepatic lateral lobectomy and exploration of common bile duct, the amylase value of bile, which was collected by the T-tube, was still obviously increased. Then the endoscopic sphincterotomy was performed, the amylase value of the bile decreased obviously and no abnormality was found in the follow-up for half a year after discharge.ConclusionsConcept and diagnostic criteria of “Japanese clinical practice guidelines for pancreaticobiliary maljunction” are conflicting and inaccurate. Severity of pancreaticobiliary reflux and change of amylase value of bile might have a more important diagnostic value. Endoscopic sphincterotomy might be suitable for a few special types of pancreaticobiliary maljunction.