ObjectiveTo explore clinical manifestation, diagnosis and treatment of IgG4 sclerosing cholangitis developed postoperative gastroduodenal hemorrhage, so as to improve awareness and treatment of this disease. MethodThe clinical data of a case of IgG4 sclerosing cholangitis misdiagnosed as the hilar cholangiocarcinoma which developed postoperative gastrointestinal hemorrhage in this hospital were analyzed retrospectively. ResultsThis patient was misdiagnosed as the hilar cholangiocarcinoma and accepted the radical resection, while the postoperative pathology proved to be the IgG4 sclerosing cholangitis. One month later, the patient developed the acute gastrointestinal hemorrhage and it was resolved by using the endovascular embolization. ConclusionsPreoperative distinguishing IgG4 sclerosing cholangitis from hilar cholangiocarcinoma can avoid an unnecessary surgery. Endovascular intervention is both a useful measure of diagnosis and treatment for gastroduodenal pseudoaneurysm. Attention should be paid to arterial protection during process of arterial osteogenesis in hepatobiliary operation.