Background: Extramammary Paget disease (EMPD) is an unusual skin neoplasm with unclear pathogenesis. The most common site of involvement is the vulva. Vulvar EMPD associated with third degree uterovaginal prolapse is very rare. Case: We report a case of a third-degree uterine prolapse complicated by an isolated vulvar EMPD and its surgical treatment. A 79-year-old woman was referred from a secondary care hospital with third degree uterovaginal prolapse and persistent vulvar itching and pain. Gynecologic examination showed a pigmented vulvar erythematous plaque, and a subcutaneous nodule in the major labia. A biopsy of the lesion revealed a vulvar EMPD. Neither internal malignancy including colorectal or genitourinary carcinoma nor distant metastasis was identified in any of the clinical examinations. She received laparoscopically assisted vaginal hysterectomy with bilateral salpingo-oophorectomy, radical vulvectomy with primary closure, and lateral suspension of vaginal vault with mesh. A histopathologic examination of the resected vulvar specimen revealed Pagetoid tumor cells that had spread through the epidermial layers without dermal invasion. She was diagnosed as FIGO stage IB vulvar cancer and did not receive any adjuvant therapy. No complications occurred during the wound healing process. Conclusion: Vulvar itching and pain associated with long standing genital prolapse need careful evaluation including punch biopsy for underlying carcinoma. We suggest surgical excision and laparoscopic suspension of vaginal vault with mesh as the optimum treatment for early stage vulvar EMPD associated with genital prolapse.