Vulvar cancer was reported in 3,200 women in 1998, resulting in 800 deaths. Recent evidence suggests that vulvar cancer comprises two separate diseases. The first type may develop from vulvar intraepithelial neoplasia caused by human papillomavirus infection and is increasing in prevalence among young women. The second type, which more often afflicts older women, may develop from vulvar non-neoplastic epithelial disorders as a result of chronic inflammation (the itch-scratch-lichen sclerosus hypothesis). Although vulvar cancer is relatively uncommon, early detection remains crucial given its significant impact on sexuality. Diagnosis is based on histology; therefore, any suspicious lesions of the vulva must be biopsied. Excisional or punch biopsy can be performed in the physician's office. Clinicians must closely monitor suspicious lesions because delayed biopsy and diagnosis are common. Once diagnosed, vulvar cancer is staged using the TNM classification system. Treatment is surgical resection, with the goal being complete removal of the tumor. There has been a recent trend toward more conservative surgery to decrease psychosexual complications.