Extramammary Paget's disease of the vulva is a rare intraepithelial neoplasm which is most commonly seen in postmenopausal Caucasian females. The most common presenting complaint is pruritis, vulvar pain, red eczematoid skin change. Treatment of Paget`s disease of the vulva requires wide local excision and if there is an underlying adenocarcinoma, radical vulvectomy with ipsilateral inguinal femoral lymphadenectomy is required. We experienced a case of extramammary Paget`s disease of the vulva, underwent radical vulvectomy with gluteal thigh flap and present with a brief review of literature.