Papillary cystic neoplasm of the pancreas is a rare entity. We report on two patients, one of them having carcinomatosis, and review the literature. This neoplasm is usually found in young women. The most common presentation is a painless abdominal mass. Abdominal ultrasound and CAT scan are helpful in defining the origin of the mass. Due to its benign behavior, the neoplasm is amenable to surgical resection with cure. Immunohistochemistry and electron microscopy should be performed for classification and definition of this curable pancreatic neoplasm.