Autoimmune hemolytic anemia and severe giant cell hepatitis is an very uncommon association. Two patients aged 5 months and 2 years developed severe anemia, jaundice, hepatomegaly and splenomegaly. Laboratory tests demonstrated autoimmune hemolytic anemia and elevated amino-transferases. Diffuse giant cell transformation and distorted architecture was seen in liver biopsy. One patient had a cytomegalovirus infection. Early steroid therapy did not prevent fatal course with liver failure in one patient.