Extramammary Paget's disease is a rare cutaneous, intraepithelial adenocarcinoma involving primarily the epidermis but occasionally extending into the underlying dermis. The condition typically presents as a red, velvety, pruritic skin rash of the vulva region which closely mimics a multitude of other, more common conditions. As a result, vulvar Paget's disease is frequently misdiagnosed, leading to an often lengthy lag time (an average of about 2-3 years) between the onset of symptoms and diagnosis.