The incidence of human papilloma virus-related vulvar intraepithelial neoplasia is increasing worldwide. This is associated with an increasing incidence of invasive vulvar cancer in young women. Undifferentiated vulvar intraepithelial neoplasia has an invasive potential; a subset of very young patients with pigmented lesions and spontaneous regression has been described. Differentiated vulvar intraepithelial neoplasia is human papilloma virus negative and affects older women, who are at risk of invasive cancer. Chromosomal changes and angiogenesis may play a role in carcinogenesis. Immunocompromised women bear a substantial risk of vulvar intraepithelial neoplasia. These facts demand the awareness of both women and physicians, because there is evidence of diagnostic delays in patients with vulvar cancer. The standard treatment is surgical excision, which may be combined with laser treatment in extensive disease. Preliminary results of topical antiviral agents and photodynamic therapy are available, but remain to be confirmed by prospective, placebo-controlled studies.