Abstract: IgG4-related disease (IRD), a multi-organ involved autoimmune disease, has been newly defined for only about 10 years. It is characterized by elevated serum IgG4, storiform fibrosis, and massive infiltration of IgG4 positive plasma cells in involved organs. Biliary tract is one of the most commonly involved organs. IgG4-related sclerosing cholangitis (IRSC) is the clinical entity of IRD in biliary tract. It always has clinical symptoms similar to biliary-pancreatic malignancy, such as localized biliary wall thickness, dilation of the bile duct, and obstructive jaundice. IRD is sensitive to the treatment of steroids with a satisfactory prognosis and does not warrant surgical treatment. However, in recent years, we met several cases that underwent an operation due to the bile duct stricture and obstructive jaundice, yet with a final pathological report of IRSC. After reviewing these cases, we found that IRSC was seldom considered before surgery. Moreover, there were some cases of biliary malignancy with elevated serum IgG4 that were misdiagnosed to be IRSC, which led to missing the best operative time. Therefore, it is of great clinical importance to widely broadcast the knowledge of IRSC and especially to deepen the understanding of IRSC for the hepatic-biliary-pancreatic surgeons.