Lichen sclerosus (LS) is a chronic autoimmune disease first described by Hallopeau in 1881. It has had various names over the years, including leukoplakia, kraurosis vulvae, balanitis xerotica obliterans, and lichen sclerosis et atrophicus, but in 1976, the International Society for the Study of Vulvovaginal Disease adopted the term lichen sclerosus. LS primarily affects the genital skin, with hypopigmentation and skin atrophy as its hallmarks. Women are more commonly affected than men, and LS can lead to phimosis or scarring of the vaginal introitus. Diagnosis is based on clinical features, but biopsy confirmation is often necessary. If left untreated, LS can result in the destruction of anatomic structures, functional impairment, and an increased risk of malignant evolution. Treatment and long-term follow-up are therefore essential.