A solid and cystic tumor of the pancreas in a 11-year-old girl is reported. The child presented with abdominal pain and vomiting. A voluminous tumor, arising from the pancreas tail, was removed with distal pancreatectomy. These neoplasma occur predominantly in girls and young women, the prognosis is usually good in spite of histologic finding of apparent malignancy. The histochemical, immunohistochemical and electron examinations of this tumor show polymorphic differentiation, which means that part of the all can differentiate into duct epithelium, acinar or endocrine line. So up to, recently this tumor has been misclassified within other pancreatic tumors, and frequently has been managed with aggressive surgery. But as the neoplasm usually behaves like a very low grade malignancy, its complete removal is the treatment of choice for the tumors arising anywhere in the pancreas. The authors emphasize the importance of accurate diagnosis and of extirpative surgery in children with pancreatic malignancy.