Pancreatoblastoma is an extremely rare pancreatic tumor in childhood, comprising 0.5% of pancreatic non-endocrine tumors. It mostly affects children of under 8 years old without special sexual predilection. Abdominal mass is the dominant clinical feature and abdominal X-ray, sonography, UGI series, CT scan are of assistance in establishing diagnosis. The presence of acinar cells with zymogen granules, squamous metaplasia and endocrine components makes the diagnosis. We report a case of 4-year-old girl, who suffered from abdominal pain for two months. Abdominal mass was found at our OPD and abdominal sonography, UGI series, CT scan all indicated a parapancreatic tumor. AFP was 4700 ng/ml. Laparotomy confirmed a pancreatic tumor and only partial excision was performed due to tumor invasion and adhesion to major vessels. Diagnosis of pancreatoblastoma was made by pathohistology, cytochemical special stains and electronic microscopic examination of the tumor. Neither chemotherapy nor radiotherapy was performed due to family refusal. Then patient followed up at NTUH OPD regularly. Unfortunately patient expired because of the regrowth of residual tumor eight months later.