Reconstruction of total vulvectomy defect represents a challenge, and reconstructive methods include skin graft, local skin flap, musculocutanous flap, and pedicled perforator flap. We report a case of a 63-year-old patient affected by extramammary Paget's disease of vulva who underwent total vulvectomy receiving reconstruction with bilateral profunda artery perforator based V-Y rotation advancement flap. The literature about vulva reconstruction was reviewed. This innovative flap design combined the classic rotation flap and V-Y advancement flap with inclusion of the profunda artery perforators to augment the flap blood supply. Besides, the internal pudendal nerve was preserved to maintain the sensation of the neo-uvula. The flap survived completely without major post-operative complications with 9-months follow-up. Profunda artery perforator based V-Y rotation advancement flap may represent a valuable option in total vulvectomy defect reconstruction. © 2015 Wiley Periodicals, Inc. Microsurgery 35:668-671, 2015.