Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are being diagnosed with increasing frequency, probably due to the widespread use of cross-sectional imaging. The natural history of these tumors is still largely unknown. Several studies have shown topographical as well as biological differences between main duct and branch duct IPMNs. In the past few years, diagnostic work-up and, in particular, management of these tumors have evolved significantly. Data support the need for resection of main duct IPMNs and the possibility of nonoperative management in selected cases of branch duct tumors. In this systematic review, we analyze the most recent publications related to IPMNs, with particular emphasis on problems related to managing this challenging disease.