Two cases with papillary cystic neoplasm of the pancreas are reviewed and discussed. Up to recently, the tumors have been misclassified as nonfunctioning islet cell tumor or carcinoma, acinar cell carcinoma, papillary cystadenocarcinoma, or pancreatoblastoma. It frequently has been managed with aggressive surgery such as pancreatoduodenectomy. The tumors are well encapsulated and the cut surfaces are characteristically solid and hemorrhagic. Ultrasonography and CT scan are the most useful tools for the diagnosis. The neoplasms usually behave like a very low grade malignancy, so complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. We have a boy and a girl who have papillary cystic neoplasm. The boy was 12 years old and the girl was 14 years old. Both underwent distal pancreatectomy and the progress were uneventful. We have a boy and a girl who have papillary cystic neoplasm. The boy was 12 years old and the girl was 14 years old. Both underwent distal pancreatectomy and the progress were uneventful.