Hepatoblastoma, the most common primary liver tumor of early childhood, is a rare neoplasm, accounting for 0.8-2.0% of all pediatric cancers. The etiology is unknown. The most common method of testing for hepatoblastoma is alpha-fetoprotein (AFP). AFP is used as a biomarker for presence of residual liver tumor, therefore indicator of the successful treatment. Multimodal therapy is composed of chemotherapy and surgical intervention. In general, the cure of hepatoblastoma in children depends on complete resection of the primary tumor. While > or =90% of patients who undergone primary complete resection of their tumors can be cured, patients with unresectable or metastatic tumors show survival rate of < or =65%. Liver transplantation (LT) is a feasible treatment option for carefully selected patients who are free of extrahepatic disease prior to LT. The still dismal outcome of multifocal disseminated tumors warrants the investigation of new cytotoxic drug and substances against specific molecular targets.