Vulvar lichen sclerosus (LS) is a chronic inflammatory disease which affects genital labial, perineal and perianal areas, producing significant discomfort and psychological distress. However there may be diagnostic delay because of late presentation and lack of recognition of symptoms.The purpose of this clinical guide is to provide advice on early recognition and treatment.Literature review and consensus of expert opinion.The etiology of LS in peri and postmenopausal women is unknown, although autoimmune, genetic and infectious factors have been implicated. Definitive diagnosis of non-malignant disorders depends on the histology of biopsied tissue. LS associated with cellular atypia should be classified as intraepithelial neoplasia. Topical corticosteroids are the most effective treatment, although prolonged treatment may be associated with dermal atrophy. Topical calcineurin inhibitors, such as tacrolimus or pimecrolimus, may be a safe and effective alternative treatment without risk of corticosteroid-related vulvar atrophy since they do not affect collagen synthesis. LS recurrences are frequent, and can lead to significant physical discomfort and emotional distress that affect mood and sexual relationships. Anatomical changes may require surgical management.