Intraductal papillary neoplasm consists a variant of the mucin-producing neoplasms of the pancreas; these are rare diseases that can be divided into mucinous ductal ectasias and intraductal papillary neoplasms. Such neoplasms have a malignant potential, and their prognosis is good if they are early diagnosed and localized surgical resection. This paper describes a case of an intraductal papillary neoplasm localized in the tail of the pancreas and treated by distal splenopancreatectomy. The literature on this rare disease is also reviewed.