A rare case of verruciform xanthoma of the vulva is reported. Diagnosis was made possible by histopathological examination and immunohistochemical staining. Verruciform xanthomas generally occur in the oral cavity. To the best of our knowledge, this is the third reported case of the tumour located on the genital mucosa. Immunohistochemical study supported the histiocytic origin of the lesion. Clinically, verruciform xanthomas may mimic other verrucous lesions of the vulva, such as seborrhoeic keratosis, verruca simplex, condyloma acuminatum, verrucous carcinoma, or erythropasia of Queyrat, or conditions such as histiocytosis, cutaneous lipidosis, or granular cell myoblastoma. The characteristics that differentiate those conditions from verruciform xanthoma, which can be seen only on histology, are given.
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