Introduction: Lichen sclerosus et atrophicus mainly affects the genital area. The palmo-plantar involvement is most often part of a diffuse cutaneous involvement, which is rarely exclusive. We report a case of acral lichen sclerosus et atrophicus, without any other involvement. Case Report: A 58-year-old man presented with lesions of the hands and feet, consisting of subungual hyperkeratosis and squamo-keratoticperi-onyxis of the toenails, sclero-atrophic ivorine plaques with depressed keratoticmicropapules on the soles and palm hollows, extending to the lateral and dorsal surfaces, with involvement of the interdigital and inter-toe spaces. There was no other cutaneous or genital involvement. Histological examination showed a lichen sclerosus et atrophicus appearance. Discussion: A few cases of lichen sclerosus et atrophicus limited exclusively to the feet and/or hands have been reported, affecting either the palmoplantar or dorsal surface, with or without ungual involvement. No case of lichen sclerosus et atrophicus affecting only the feet and hands on both surfaces with onychodystrophy, as in our case, has been reported.