Long-term functional results after standard pancreatic resections are a major concern, especially in children. An alternative pancreas-sparing procedure (such as central pancreatectomy) should be taken into consideration whenever it is feasible, and a prolonged survival is expected. Pancreatoblastoma is an unusual malignant tumor in childhood. However, in initially resectable tumors, the 15-year survival is more than 80%. Thus, there is a potential role of a conservative pancreatic resection in successful treatment of pancreatoblastoma. The management in a case of a 16-year-old girl with a pancreatoblastoma in the body of pancreas is presented. Complete surgical resection by central pancreatectomy followed by chemotherapy led to a prolonged disease-free survival, with good functional results. Central pancreatectomy could be an alternative surgical technique in some selected cases of pancreatoblastoma, having the lowest incidence of postoperative exocrine and endocrine insufficiency rate and offering the best nutritional status.

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