ObjectiveTo explore the clinical characteristics of pancreaticobiliary maljunction (PBM) and its disease spectrum, and to evaluate therapeutic endoscopic retrograde cholangiopancreatography (ERCP). MethodsData of 52 PBM patients who received therapeutic ERCP procedures for abdominal pain, jaundice and fever in Department of Gastroenterology of Peking University Third Hospital from June 2006 to March 2021 were collected. The clinical characteristics, typing, the change of disease spectrum and ERCP procedures were analyzed. ResultsAmong 52 PBM patients, female was more common. Abdominal pain and jaundice were the most common clinical manifestations, among which 20 were type Ⅰ, 25 type Ⅱ and 7 type Ⅲ. Half patients had the choledochal cyst. The mean timespan from the first onset to the final diagnosis was 12.2 years. Twenty-four cases （46.2%） had changes in PBM disease spectrum. Among 69 ERCP procedures, 5 （7.2%） failed. Difficult cannulation rate was 34.6% (18/52)，and 11 patients underwent advanced cannulation techniques, while it was 15.4% (657/4 275) in the conterpart non-PBM patients in the same period, with significant difference between them (χ2=14.455，P<0.05). Multiple therapeutic ERCP techniques including endoscopic sphincterotomy, pancreatic stent placement, removal of stones from the duct were applied with the successful rate of 92.8% （64/69）. The incidence of post-ERCP pancreatitis was 15.4% (8/52). ConclusionThe chief clinical problem may be changed over time in PBM patients. Although ERCP plays an important role in PBM and its disease spectrum, there may be a higher rate of difficult cannulation and postoperative complications.