Carcinoma of the vulva is an uncommon malignant neoplasm (approximately one-fifth as frequent as cervical cancer) and represents 4% of all genital cancers in women. Approximately two-thirds of cases occur in women older than 60 years, and squamous cell carcinoma is the most common histologic type. Several different subtypes of squamous cell carcinoma have been described in the vulva; however, in terms of etiology, pathogenesis, and histologic features, most carcinomas belong to one of two categories: keratinizing squamous cell carcinomas associated with chronic inflammatory skin disorders, and basaloid or warty carcinomas related to infection with high oncogenic risk human papillomaviruses. Glandular neoplasms of the vulva arise from the vulvar apocrine sweat glands (papillary hidradenoma and Paget disease) or the Bartholin gland and their cause is not known.