The question of whether vulvar Paget's disease is an intraepidermal disease with potential to invade the underlying dermis or a tumor of adnexal origin with spread to the overlying epidermis remains to be resolved. The most common complaint is pruritus. The lesion is described as red, inflamed or erythematous. The delay in diagnosis is considerable in most cases. The disease can be diagnosed only by biopsy. The lesion frequently extends beyond the clinically apparent borders. The disease without underlying invasive carcinoma appears to remain as an intraepithelial malignancy for a long time. The high incidence of other malignancies (breast, colon, cervix, ovary) warrants careful examination in patients with Paget's disease of the vulva. Close follow-up for a longer period of time will be necessary before excluding recurrence. A major goal of conservative surgery is to preserve sexual function, normal anatomy and body image.