Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are rare tumors with different characteristics than conventional ductal pancreatic adenocarcinomas. Although they are commonly classified as cystic neoplasms of the pancreas, within their own subgroup of pancreatic cystic tumors, they are unique in their presentation, histologic characteristics, treatment, and survival rates. Currently, strategies are being implemented to better characterize these tumors preoperatively. Once IPMN is diagnosed, treatment strategies are based upon multiple factors, including patient condition, symptoms, and type and extent of disease. Although these factors may determine different treatment strategies, surgery remains the mainstay of therapy due to the favorable survival rate if the disease is diagnosed and treated prior to the development of invasive carcinoma. The goal of treatment is to alleviate symptoms and to extirpate disease prior to its transition to malignancy, invasion, and metastases. Although some experts advocate a nonoperative approach to patients with suspected benign disease, the risk of progression to malignancy can present a dilemma for the treating physician and patient. Unfortunately, differentiation of benign from malignant disease can only be determined conclusively following complete review of the entire surgical specimen. To further complicate treatment strategies, IPMN is a multifocal disease, and additional lesions can develop in the remnant pancreas. This fact has compelled most physicians familiar with the disease to institute lifelong surveillance for patients with the disease. Although our understanding of IPMN has increased greatly since its initial description in 1982, the natural history of the disease is poorly defined, and there is no consensus among experts on standards of practice. Although additional long-term follow-up of greater numbers of patients and their response to various interventions are necessary to develop consensus-based practice guidelines, this review will discuss our treatment recommendations based upon a review of the literature.