Papillary cystic neoplasm of the pancreas is rare low grade malignant tumor, especially in children, and that occurs chiefly in young women. This type of pancreatic tumor has more benign clinical features than any other type of the tumor of the pancreas dose, and long survival or even cure can be expected by aggressive treatment. We present a 10-year-old frmle patient with a papillary-cystic neoplasm of the pancreas, she had distant metastatic evidences to the liver, omentum and peritoneum. The tumor was totally excised with the spleen and greater omentum, and chemotherapy was carried out with vincristine and 5-FU. On the follow up studies, there are neither evidence of recurrence nor evidence of disease and the child has been doing well for two years.
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