Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are now a well-recognized category of slowly growing tumors with a remarkably better prognosis, even when malignant, than pancreatic ductal adenocarcinoma. Their clinical and pathohistologic features have been increasingly attracting the attention of clinicians since their first description 25 years ago. Despite its burgeoning volume recently, accumulated literature devoted to IPMN still provides a low level of evidence with regard to diagnosis, treatment, and prognosis. Therefore, we performed a Medline-based systematic review of the literature aimed at clearly defining the clinicopathologic characteristics of pancreatic IPMN and determining the best currently available evidence-based principles of diagnosis and management of patients with this disease.