To report five cases of isolated IgG4-related sclerosing cholangitis (IAC) and to summarize their clinical characteristics and the differential diagnosis.The clinical data of five patients with isolated IAC were retrospectively analyzed, including laboratory tests, imaging examination and liver pathology. Their treatment and prognosis were also discussed.All five patients had no history of pancreatitis. All five patients presented with jaundice and three of them had fluctuant jaundice. The serum IgG4 levels was increased in all five patients. The images study showed bile duct stenosis in all 5 patients (2 in hilar bile duct, 2 in intrahepatic bile duct and 1 in hilar associated distal bile duct). The enlargement of pancreas was found in 2 patients. Liver pathology revealed fibrosis around small bile duct and IgG4-positive plasma cells infiltration in all 5 patients. Two patients underwent surgical procedure without relief. All five patients were relieved after the treatment of steroid. All patients were followed up from 6 months to 2 years and no recurrence was detected.Isolated IAC is a rare disease and it could be misdiagnosed as cholangiocarcinoma. The surgical procedure has the limited effect for the treatment of IAC and it should be avoided. IAC should be considered for patients with fluctuant jaundice of long history and enlargement of pancreas on imaging. Serum IgG4 test and liver biopsy are helpful for the diagnosis of IAC.