Autoimmune cholangiopathy (IgG4-associated sclerosing cholangitis) can mimic symptoms and signs of many other diseases and thus it remains often undiagnosed or misdiagnosed. The definitive diagnosis is based on pathological findings. Thus, only very few patients have been reported in the literature. We report a case of a 64-year-old female with autoimmune cholangiopathy (IgG4-associated sclerosing cholangitis) that had presented mass-like lesions at liver parenchyma and occlusion of the common bile duct and performed a metal stent inserted in the common bile duct for diagnosis of cholangiocarcinoma in another medical center 5 years ago. High levels of the IgG4 of the patient and pathologic findings of mass-like lesion specimen disclosed severe infiltrations of lymphocytes and plasma cells and suggested an autoimmune cholangiopathy (IgG4-associated cholangiopathy). The patient was treated with prednisolone. The narrowing of the common bile duct, mass-like lesions at liver parenchyma and complaints of the patient improved significantly 1 month after corticosteroid treatment.