Verruciform xanthoma is a rare, benign, mucocutaneous, nondestructive lesion characterized by proliferation of non-Langerhans lipid-rich histiocytes. We describe the clinical and pathologic findings in a 30-year-old female with recurrent verruciform xanthoma of the vulva 8 years after initial therapy. The differential diagnosis includes seborrheic keratosis, verruca simplex, condyloma acuminatum, granular cell myoblastoma, vulvar intraepithelial neoplasia, bowenoid papulosis, erythroplasia of Queyrat, and verrucous carcinoma.