Extramammary Paget's disease is a rare neoplasm that usually develops in apocrine gland-bearing areas. Paget's disease of the vulva is a rare form of extramammary Paget's disease affecting postmenopausal women. We report the case of a 39-year-old woman who presented with a vulvar lesion suggestive of Paget's disease involving only the labia majora and minora. Biopsy confirmed the diagnosis of non-invasive Paget's disease. Extension examination was negative. The patient underwent a superficial vulvectomy with healthy margins on histopathology. Postoperative management was uncomplicated.
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