Four children with major hepatoblastoma were treated with extensive surgical excision and chemotherapy. Two right lobectomies and two left extended hepatectomies were performed. The first patient only received postoperative chemotherapy. The others three had preoperative chemotherapy for unresectable hepatoblastoma. All these children exhibited an initial response with marked reduction in size of their primary tumor. The only child who died during operation had disease involving both lobes of the liver, inferior vena cava and lungs metastasis. The follow up of the three other children is 26 to 32 months. Two of them are free of all disease. The third had lung isolated metastasis removed and is actually free of disease with follow up of 32 months. Authors discuss chemotherapy interest and limits in major hepatoblastoma.