Abstract: The clinical and pathological data of 4 cases with acantholytic dermatosis in genital region were collected and relevant literature was reviewed. All the patients were female, aged from 35 to 48 years old. The clinical manifestations were single or multiple papules around the vulva or anus. Histopathological examination revealed epidermal hyperkeratosis, acanthosis and acantholysis. The patients were treated by laser or surgical excision and no recurrence after 0.5-2 years follow up.