Lichen sclerosus (LS) is an inflammatory immune-mediated disease that affects anogenital area and is prone to progression and chronic course. LS belongs to atrophic skin disease, not being a form of localized scleroderma, according to the International Classification of Diseases. Dermatosis is most common in both premenopausal and postmenopausal women, as well as in those of reproductive age. Vulvar lichen sclerosus (VLS) significantly affects patients’ life quality due to pronounced itching, sexual dysfunction, anatomical changes caused by scarring and in some cases malignization. The etiology and pathophysiology of disease is unclear until the present. Autoimmune, hormonal and infectious hypotheses, as well as chronic stimulation and traumas theories under consideration require conclusive evidence. Clinically, the disease is often not recognized timely by specialists, leading to delayed treatment and complications. The use of topical glucocorticosteroids (tGCs) combined with emollients is the main treatment method, resulting in improvement on average in 80-90% of cases, despite the lack of accurate data on the etiopathogenesis. The current data on the causes of LS development, triggers, clinical features and diagnosis are considered, as well as therapy methods are discussed.