Histiocytosis X (HX) is a rare disorder of Langerhans cells and most commonly occurs in children. We report a case of HX of the vulva in a 76-year-old woman that clinically simulated a yeast infection of the vulva but histologically resembled an amelanotic melanoma. We briefly report the clinical and pathologic features of this case, which responded to vincristine followed by vinblastine and was in complete remission after nine months.