Recently, liver transplantation (LT) has been increasingly performed for unresectable hepatoblastoma (HB) with acceptable results. Anatomic resection or transplantation following adequate chemotherapy for PRETEXT III/ IV patients has been recommended for optimal outcomes.

However, hepatoblastoma with extrahepatic involvement (such as lung and peritoneal dissemination) or tumor thrombi extending into portal vein/ inferior-vena-cava is challenging. The meticulous surgical approach is technically demanding and might require major liver resection or transplantation with vascular reconstruction and extracorporeal circulation.

We herein report cases with liver transplantation for advanced hepatoblastoma using extracorporeal circulation with extended tumor thrombi into the right atrium. Patient selection, preoperative evaluation of the anatomy, and intraoperative assessments are essential to achieve sufficient outcomes. Our findings justify further investigation to identify the optimal hepatectomy or transplant management for children with HB.