Pancreatoblastoma is a rare neoplasm encountered most commonly in infants and young children. Unresectable or metastatic tumors have a poor prognosis despite adjuvant chemotherapy or radiotherapy. We report the successful use of CT-guided radiofrequency ablation subsequent to intensive chemotherapy in an 8-year-old girl with liver-metastasizing pancreatoblastoma and with right and segment 4 portal vein tumor thrombosis. She has been in remission for 3 years.