Localized perineal papules and plaques with acantholytic histology is characterized clinically by discrete and grouped papules, which are localized to the perineal or perianal areas. Histologically it shows focal suprabasal acantholysis. A 51-year-old man was referred to our department for verrucous skin lesions confined to perineal and perianal areas. Histopathologic findings of verrucous papule showed severe intraepidermal acantholysis. Although we do not know the precise nature or the incidence of this disease, localized perineal papules and plaques with acantholytic histology should be included in the differential diagnosis of verrucous papules in perineal or perianal areas.