Pancreatoblastoma (PB) is extremely rare. A 11-year-old boy, who had excision of dilated common bile duct with hepaticoduodenostomy when 9 years old was referred to our institute for further management of abdominal pain and steatorrhea. Imaging studies showed a solid 4 cm tumor in the head of the pancreas and two lesions in the liver. Needle biopsies diagnosed PB with liver metastases. After five courses of chemotherapy, the primary tumor was completely resected with pancreaticoduodenectomy (PD) and Child's pancreaticobiliary tract reconstruction. The liver metastases were resected. Postoperative recovery was uneventful. Here, we present this case and review the literature focusing on management of PB.