Pancreatoblastoma is a rare pediatric pancreatic malignancy. It confers a worse prognosis if there is metastatic or unresectable disease. We report a case of a 12-year-old boy who presented with pancreatoblastoma of the head of the pancreas with multiple intrahepatic metastases. We successfully treated him using aggressive chemotherapy, multi-stage surgery, and gemcitabine chemotherapy, which has not been described as treatment for pancreatoblastoma. Rare childhood malignancies such as pancreatoblastoma require a coordinated, multidisciplinary approach and frequent reassessment of therapeutic interventions. Complete surgical resection is important for cure and may require complex, aggressive, multiple-stage surgical procedures with concurrent or novel chemotherapy.