Brownstein described a previously unrecognized benign acanthoma, the "Acantholytic acanthoma" in 1988. We report a rare case of acantholytic acanthoma on the right labia minor of vulva in a 34-year-old woman. Due to the rarity of this lesion in the female genitalia, it may be confused with other lesions more commonly affecting this region. Correct diagnosis of acantholytic acanthoma may result in proper treatment of the patient.