Intraductal papillary mucinous neoplasms (IPMNs), characterized by intraductal papillary growth and thick mucin secretion, have increasingly been recognized. Despite modern preoperative evaluation, major difficulties still remain in distinguishing malignant invasive types from benign IPMNs. Following a PubMed database search, all relevant abstracts and articles on IPMN published in English and Chinese were reviewed. Main-duct and the mixed type IPMNs carry a higher risk of malignancy as compared with branch-duct type IPMNs. Treatment of branch-duct type IPMNs remains controversial. Once operation is indicated, intraoperative frozen section of margins plays an important role in the decision concerning the extent and type of surgery. Pancreatectomy, partly preserving both endocrine and exocrine pancreatic function, is advocated for most patients with IPMN, though total pancreatectomy may be necessary in some. Both for patients subjected to surgery and those only observed, IPMN patients need regular close follow-up to identify recurrence or progressive disease. Eur J Gastroenterol Hepatol 22: 1029-1038 (C) 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins.