Giant cell hepatitis(GCH)with autoimmune hemolytic anemia is a rare entity,limited to young children,with an unknown pathogenesis.We report the case of 9-mo old who presented with fever,diarrhea and jaundice four days before hospitalization.Physical examination found pallor,jaundice and hepatosplenomegaly.The laboratory workup showed serum total bilirubin at 101 μmol/L,conjugated bilirubin at 84 μmol/L,hemolytic anemia,thrombocytopenia and immunoglobulin G(IgG)and anti-C3d positive direct Coombs’ test.The antinuclear,anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies,antiendomisium antibodies were negative.Serological assays for viral hepatitis B and C,cytomegalovirus,herpes simplex and Epstein Barr virus were negative.The association of acute liver failure,Evan’s syndrome,positive direct Coomb’s test of mixed type(IgG and C3)and the absence of organ and non-organ specific autoantibodies suggested the diagnosis of GCH.The diagnosis was confirmed by a needle liver biopsy.The patient was treated by corticosteroids,immunomodulatory therapy and azathioprine but died with septicemia. Giant cell hepatitis (GCH) with autoimmune hemolytic anemia is a rare entity, limited to young children, with an unknown pathogenesis. We report the case of 9-mo old who presented with fever, diarrhea and jaundice four days before hospitalization. Physical examination found pallor, jaundice and hepatosplenomegaly.The laboratory work showed showed serum total bilirubin at 101 μmol / L, conjugated bilirubin at 84 μmol / L, hemolytic anemia, thrombocytopenia and immunoglobulin G (IgG) and anti-C3d positive direct Coombs’ test.The antinuclear, anti-smooth muscle and liver kidney microsomes 1 non-organ specific autoantibodies, antiendomisium antibodies were negative. Serological assays for viral hepatitis B and C, cytomegalovirus, herpes simplex and Epstein Barr virus were negative. The association of acute liver failure, Evan’s syndrome, positive direct Coomb’s test of mixed type (IgG and C3) and the absence of organ and non-organ specific autoantibodies the diagnosis of GCH. The diagnosis was confirmed by an eedle liver biopsy.The patient was treated by corticosteroids, immunomodulatory therapy and azathioprine but died with septicemia.