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Background: Ectopic breast (extramammary) fibroadenomas of the vulva are rare, cosmetically disfiguring, and very difficult to distinguish from other labial masses on physical examination. Extramammary tissue is susceptible to similar benign and malignant changes seen in the breast. Some patients with ectopic breast lesions may harbor an underlying urinary tract anomaly., Case: We report a case of a young woman who presented with an unknown progressively enlarging mass in the vulva. The mass was excised completely in a cosmetic manner and histology demonstrated an ectopic breast fibroadenoma., Conclusion: Clinicians should include extramammary lesions in their preoperative differential diagnosis of vulvar masses since some may become neoplastic. Treatment is complete excision and renal imaging should be considered after a confirmed histopathologic diagnosis.