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We report a case of a 63\u2010year\u2010old patient affected by extramammary Paget's disease of vulva who underwent total vulvectomy receiving reconstruction with bilateral profunda artery perforator based V\u2010Y rotation advancement flap. The literature about vulva reconstruction was reviewed. This innovative flap design combined the classic rotation flap and V\u2010Y 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\u2010uvula. The flap survived completely without major post\u2010operative complications with 9\u2010months follow\u2010up. Profunda artery perforator based V\u2010Y rotation advancement flap may represent a valuable option in total vulvectomy defect reconstruction. \u00a9 2015 Wiley Periodicals, Inc. 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