Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy accounting for approximately 1% of vulvar cancers. The rarity of this disease has caused difficulties in its characterization. Controversies exist in the literature regarding many aspects of this condition including the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. This extensive review takes a closer look at what is known about EMPD and the conclusions that have been drawn from this information. This article also provides a practical approach to patients with EMPD. Copyright © 2010 Elsevier Inc. All rights reserved.