A 10-month-old white infant presented with abdominal distention and bilateral scrotal hernia. Imaging studies of the abdomen and thorax showed a huge liver with multiple tumor masses and calcification involving all the segments. There was thrombosis in the inferior vena cava and right atrium. alpha-Fetoprotein was 246,000 IU/mL. HB-94 chemotherapy protocol was started at once due to rapid deterioration of the patient. Surgical biopsy performed after the first IPA (ifosfamide, cisplatin, doxorubicin) course showed hepatoblastoma with macrotrabecular variant. After a second IPA course and 2 courses of carboplatin and etoposide, the boy's clinical condition was excellent with normal alpha-fetoprotein but minimal regression and increased calcification in the tumor mass. Hepatic tumor was unresectable and no surgical intervention was performed. Transplantation could not be performed because of high morbidity and mortality. Despite general agreement that complete surgical resection is the cornerstone of treatment for patients with hepatoblastoma, the patient is in remission with 100% Karnofsky score in the 43 months of diagnosis.