This review addresses recent and important advances in our knowledge of several uncommon or rare disorders of the vulva including Paget's disease, vulva intraepithelial neoplasia, lichen sclerosus and squamous hyperplasia and their relationship to squamous carcinoma. Emphasis is placed on the two biologically different types of squamous carcinoma related and unrelated to human papillomavirus infection. Finally, the relatively recent concept of the sentinel node as applied to vulva carcinoma is discussed.