Idiopathic granulomatous vulvitis is an uncommon anogenital area disease described in the last few decades. It causes an inflammatory reaction that culminates in swelling and possibly distortion of the female anogenital area. Many reported cases consider this non-infectious entity the genital counterpart to granulomatous cheilitis. We here present a 64 years old female patient with idiopathic granulomatous vulvitis co-existing with lipodermatosclerosis, with the excellent response of the former condition to hydroxychloroquine and potent topical steroids.