Hepatoblastomas are the most frequent malignant liver tumors of childhood. Prognosis mostly depends on their resectability. Unresectable tumors are almost invariably fatal, unless their size can be decreased by chemotherapy and/or irradiation to permit surgical removal. In the present case report we discuss the clinical course of a 15 months old boy, who was admitted with primary inoperable teratoid hepatoblastoma. After alternating intravenous and intraarterial chemotherapy as well as radiation therapy, the tumor could be successfully resected. Specific problems of histological diagnosis and of the therapeutic management are discussed, and an overview of the recent literature in behalf of the histological basis, therapeutic possibilities and prognostic factors of hepatoblastomas is given.