Since the first description of pancreatoblastoma as a malignant pancreatic tumor of childhood in 1957, approximately 200 cases have been reported. We describe an 18-year-old boy who presented with pain and jaundice and was found to have an abdominal mass. The patient initially presented with abdominal pain. During laparotomy, a 10 x 8 x 8-cm3 tumor was discovered in the pancreatic body and tail, and with 3 cystic masses, 15, 10, and 8 cm in diameter, respectively, involving the right lobe of the liver. Pathologic examination of the resected tumor revealed findings characteristic of pancreatoblastoma. The tumor formed acinar and glandular structures and solid areas and contained many "squamoid corpuscles," a defining feature of pancreatoblastoma. In spite of adjuvant chemotherapy with Adriamycin and gemcitabine, the patient returned 11 months later with several large hepatic masses, invading the pancreatic head and enlarged tracheobronchial lymph nodes. Radiotherapy, transcatheter arterial embolization therapy, and chemotherapy were performed. Unfortunately, the patient died 26 months later from disseminated tumor progression. A review of the literature reveals that pancreatoblastoma in childhood must be considered malignant but usually has a favorable prognosis in contrast to pancreatic neoplasms in adult patients. Biologic study will investigate the molecular biology of this rare tumor. The biology may help define prognosis and therapy for this kind of tumor.