RESUMO
Extramammary Pagets disease(EMPD) of the vulva is an uncommon neoplasm which accounting for 2.5% of all vulvar malignancies and 0.02% of all female cancer. Several hundred cases have been reported worldwide since the first description by sir James Paget in 1874, It generally affects postrnenopausal age poup and presents high rate of recurrence but the precise incidence remines unclear, Although Paget's disease of the breast and the vulva are histologically similar, their histogenesis and biological behavior are considered to be significantly different and EMPD has gcnerally perceived rnore benign compared to that of the breast. Four histologic forms of vulvar EMPD have been recognized and treated vulvar EMPD in according to the 4 histologic classification. The high rate of recurtence disease remains a challenge for optimal management. Recently we experienced a case of Paget's disease of the vulva, diagnosed as an intraepithelial Pagets disease preoperatively and underwent radical vulvectomy and superiomedial thigh(SMT) flap, and present with a brief review of literature.