IgG4-related disease (IgG4-RD) a newly established immune-mediated condition, can affect virtually one or more organs. The disease shows increased serum and tissue IgG4 levels. On histological evaluation, it is characterized by the presence of lymphocytes and plasma cells, storiform-type fibrosis, and obliterative phlebitis. We present a case of obstructive jaundice with imaging findings that resembled cholangiocarcinoma. The patient was diagnosed with IgG4-RD by pathological examinations. IgG4-RD often mimics malignancy, and clinical physicians should be aware of this disease in the differential diagnosis in order to optimize the treatment.