Objective To summarize the research progress of intraductal papillary mucinous neoplasms (IPMNs). Method The domestic and international published literatures related to IPMNs in recent years were collected and reviewed． Results Based on the site of tumor involved, IPMNs were classified into main duct, branch duct, and mixed types. According to the histologic features, IPMNs were divided into intestinal, pancreatobiliary, gastric, and oncocytic types. The pathological and clinical presentations of IPMNs were vary, and multiple imaging approaches including endoscopic retrograde cholangiopancreatography (ERCP), endoscopic ultrasonography (EUS), CT, magnetic resonance cholangiopancreatography (MRCP) combined with MRI, could display morphologic and functional characteristics of IPMNs. In additon, the risk of malignancy in IPMNs could be evaluated and the functions of some genes (including KRAS, BRAF, GNAS, and so on) in the development process of IPMNs had been confirmed. Conclusion IPMNs is a kind of disease different from other tumors of the pancreas in clinicopathologic features, imaging performance, and molecular biology changes, and how to link the above three aspects needs to be further studied.