Two cases of papillary and cystic neoplasm of the pancreas (PCN) occurring in 17 and 21 year old women are reported with ultrastructural and immunohistochemical findings. A review of the English literature shows that although potentially malignant, PCN, which occurs mainly in young women, is amenable to surgical cure. The significance of large PAS positive and alpha‐1‐antitrypsin positive tumour granules; the lack of specificity of alpha‐1‐antitrypsin for pancreatic acinar cells; the possibility of acinar differentiation of PCN; and its separation from pancreaticoblastoma are di