Pancreatoblastoma is an extremely rare pancreatic tumor of childhood. We report our experience in 2 patients with pancreatoblastoma who had long-term survival and review the literature with a focus on chemotherapy in pancreatoblastoma with vascular invasion. The cases were 7-year-old and 4-year-old girls who complained of an abdominal mass without jaundice. Laboratory findings including serum alpha-fetoprotein were within normal limits. Radiologic investigations revealed a mass in the pancreas with vascular invasion. After surgical resection, postoperative chemotherapy included cisplatin, doxorubicin, ifosfamide, and etoposide. They have now been well for 7 years without recurrence.