History and Objectives: Pancreatoblastoma is a rare pancreatic disease. Due to discrepancies as to the origin, histogenesis and type of treatment of the disease, the present article presents a case of pancreatoblastoma of a 10-year-old boy admitted to the Shaheed Beheshti hospital and underwent surgical operation.

Presentation of the case: A 10-year-old boy was presented with abdominal pain for 20 days prior to hospitalization. The pains were more pronounced at night and with ingestion of food. A mass of about 10cm was palpable at the epigaster region. The patient underwent the surgical operation and pathologic findings indicated pancreatoblastoma.

Results: Pancreatoblastoma, although is a malignant tumor, with early diagnosis and with prompt surgical operation, half of children recover fully.