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Hepatic veno-occlusive disease (VOD), also called sinusoidal obstruction syndrome, is rapidly progressing and involves life-threatening complications that can occur in patients receiving chemotherapy and/or bone marrow transplantation. No completely satisfactory treatment strategies have yet been established. We present a case of rhabdomyosarcoma in a 21-month-old boy who developed pancytopenia, dyspnea, jaundice, massive ascites and body weight gain of more than 10% after receiving conventional chemotherapy. Hepatic VOD was diagnosed. He recovered after supportive care and treatment with high-dose methylprednisolone.