Intraductal papillary mucinous neoplasms (IPMNs) are rare tumours rising from the pancreatic duct epithelium. They are characterized by intraductal papillary growth and thick mucin secretion; mucin fills the Wirsung and/or branch pancreatic ducts and may cause ductal dilatation. IPMNs are classified into three types, according to the site of involvement: main duct type, branch duct type, and combined type. Most branch type IPMNs are benign, while the other two types are frequently malignant. Recent advances in diagnostic imaging have led to an increased frequency of diagnosis of IPMNs, but the clinical features of them can range broadly from benign, borderline, and malignant non-invasive to invasive lesions, and their management has not yet been clearly defined. The most of patients are asymptomatic. The possibility of malignancy is increased in cases which large mural nodules are presented. Presence of a large branch type IPMN and marked dilatation of the main duct indicate the existence of adenoma. Not infrequently, synchronous or metachronous malignancies may be developed in various organs. Endoscopic retrograde cholangiopancreatography (ERCP), endoscopic ultrasonography (EUS), intraductal ultrasonography, and magnetic resonance cholangiopancreatography (MRCP) are the most valuable imaging techniques for diagnosis of these lesions. Prognosis is excellent after complete resection of benign and non-invasive malignant IPMNs. Total pancreatectomy should be reserved for patients with resectable but extensive IPMN involving the whole pancreas; its benefits must be balanced against perioperative risks.