This review generalizes the literary data on the etiology, pathogenesis and treatment of lichen sclerosus of the vulva. In the structure of gynecological pathology proportion of patients with trunk is about 7 %. Long term course of the disease, accompanied by debilitating itching can lead to neuropsychiatric and vegetative-vascular disorders, as well as violations of patients' relationships with others. There are known neurogenic, endocrine, metabolic, infectious, autoimmune and some other development theories of lichen sclerosus of the vulva. Due to the nature of pathogenetic mechanisms in the development of lichen sclerosis of the vulva more and more researchers are inclined to the necessity of introduction of topical corticosteroids in the medical practice.