Children with acute lymphoblastic leukemia (ALL) frequently present with hepatomegaly and mild liver functional impairment. Severe jaundice is rarely seen as a presenting feature. Such patients often present diagnostic dilemmas and therapeutic difficulties. Here we report a 15-year-old boy presenting with severe jaundice and hyperferritinemia, whose bone marrow smear showed B-lineage precursor ALL. We treated him with intravenous immunoglobulin, steroid, and etoposide; then his condition improved. ALL should be considered as a possible diagnosis in severely jaundiced children. Steroid and etoposide can be used as first aid when many chemotherapeutic drugs are contraindicated.