Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AHA) is a rare disease of infancy, characterized by presence of both Coombs positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Its exact mechanism is yet to be determined, but B-cell mediated autoimmunity has been proposed. Unlike ‘classical’ autoimmune hepatitis, steroid and azathioprine failed to prove efficacy. Prognosis is poor despite aggressive therapy, but promising results may await with early application of anti-CD20 monoclonal antibody, rituximab. Here we present the 7-month-old Korean male infant who initially presented with autoimmune hemolytic anemia, followed by severe hepatitis. He presented with jaundice, pallor, and red urine. Physical examination showed icteric sclerae, and splenomegaly. Laboratory findings revealed warm autoimmune hemolytic anemia with hepatitis with cholestasis. His total bilirubin level peaked at 15.6 mg/dL, his AST and ALT levels were 1781 IU/L and 4136 IU/L respectively. Liver biopsy showed giant cell transformation of hepatocytes. Hemolytic anemia and hepatitis with giant cell formation of hepatocytes, with absence of infectious, metabolic, and other autoimmune causes suggested the diagnosis of GCH with AHA. He was initially treated with intravenous methylprednisolone, followed by high dose oral prednisolone and ursodeoxycholic acid. During steroid tapering, severe hepatitis progressed, which did not respond to increased steroid dose and Intravenous immunoglobulin. His liver function tests improved after treating with rituximab, and he was successfully treated with 4 doses of rituximab.