Veno-occlusive disease (VOD) of the liver is a life-threatening state generally occurring as a complication of bone marrow transplantation or chemotherapy for Wilms' tumor. Veno-occlusive disease after standard dose chemotherapy in malignancies other than Wilms' tumor is rare and only a few cases have been published in children. We report a 19 month-old-girl with medulloblastoma who experienced fatal VOD of liver after only one course of chemotherapy including carboplatin, vincristine and CCNU for medulloblastoma. As our knowledge, this is the first report of VOD after standard dose chemotherapy for brain tumor in childhood.