Lichen sclerosus (LS) is a lymphocyte-mediated, chronic, inflammatory dermatitis which most commonly involves the anogenital area. Treatment options include topical corticosteroids, tacrolimus, carbon dioxide laser ablation and, as a last resort vulvectomy. The objective of this study is to report a refractory anogenital LS case and describe the use of a V-Y advancement flap for reconstruction of the perineal defect after vulvectomy. A case of persistent LS, treated succesfully with surgery is presented and the literature is reviewed. The patient tolerated the surgical procedure well and a satisfactory cosmetic result was obtained. A multidisciplinary team is ideal in the management of patients with persistent LS. Surgical management of LS should be reserved as a last resort for patients who are refractory to other medical treatment options and, in the case of a surgical attempt, techniques for reconstruction of the vulva should be well known.