The vulvoperineal region is key to the urinary and reproductive systems and closely related to the gastrointestinal system. Most vulvar defects are resulted from tumor ablation. Therefore, the reconstruction after oncological resection remains challenging as it aims to restore adequate function with an ideal aesthetic result. We report a case of a 46-year-old female with extramammary Paget’s disease of the vulva. She underwent oncological excision for vulvar reconstruction. Keystone perforator island flaps were performed in this patient and it demonstrated a symmetrical bilateral result of vulva subunits. The patient was satisfied with the vulva appearance and no limitations in daily activities was reported. The use of keystone perforator island flaps is a superior option for vulva reconstruction to achieve a symmetrical outcome. © 2020 by Nova Science Publishers, Inc.