Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatitis affecting primarily vulvar, perianal and perineal skin. Extragenital lichen sclerosus is most common on the neck, shoulders, and upper portion of the trunk. It is generally asymptomatic or is occasionally pruritic and presents as flat, white, polygonal papules and atrophic white plaques. It is primarily seen in postmenopausal women, but also men and children can be affected. The aetiology is not clearly established, although there is a strong association with autoimmune diseases. Morphea may coexist with LSA. Standard treatment includes topical corticosteroid and calcineurin inhibitors, such as tacrolimus. The association between LSA and squamous cell carcinoma (SCC) is confirmed and has been described chiefly in females. The risk of SCC of the vulva has been estimated to about 5% in women with LSA.