Pancreatoblastoma (PB) is a rare malignant neoplasm. PB is frequently detected in children under 10 years of age. Symptoms are nonspecific. When diagnosed, most tumors are enlarged (> 5 cm). Abdominal pain is often the first complaint (44%). Alpha-fetoprotein (AFP) levels are high. Provides long-term survival with surgical resection. It has been reported that the prognosis is poor if metastases are detected. Here we present a seven-year-old female PB case. She applied with the complaint of abdominal pain. On physical examination, a mass was palpated in the epigastric region. The changed laboratory findings were an increased serum AFP level of 171.1 micrograms/L (normal range 0-9 micrograms/L). Abdominal computed tomography (CT) examination revealed a solitary mass of approximately 6 × 4 cm in the tail of the pancreas. Multiple mass lesions were observed in the liver. These lesions were evaluated as compatible with metastasis. She was diagnosed with PB histopathologically after Tru-cut biopsy. Pathologically increased Fluorodeoxyglucose (FDG) uptake (SUVmax: 9.99) was observed in the mass lesion around the right upper quadrant gastric corpus in F-18-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography (18F-FDG PET/CT). Malignant hypermetabolic metastatic multiple hypodense mass lesions (SUVmax:7.7) were seen in the liver. OPEC chemotherapy was given. In the evaluation performed after 5 cycles of chemotherapy, a decrease in FDG uptake 18F-FDG PET/CT was detected. The patient was evaluated as responsive to treatment. This case report may contribute to the literature with its rarity and treatment approach.