Abstract:Vulvovaginal-gingival syndrome (VVGS) is a chronic mucocutaneous disease of unknown etiology, which consists of a triad of symptoms: vulvar, vaginal and gingival of lichen planus (LP) lesions. The evidence suggests an autoimmune response, a genetic and probably hormonal component. Associated factors observed in LP include stress/anxiety, hepatitis C virus (HCV) and autoimmune diseases, among others. The most serious complication of oral and possibly also vulvar Lichen Planus is the development of squamous cell carcinoma. Corticosteroids are the mainstay treatment for LP in order to control inflammatory activity. In case of failure, immunosuppressive agents can be used (Tacrolimus and Cyclosporine). We present an illustrated case of VVGS who over the years developed tongue cancer and, 10 years later, a vulvar cancer. Oral Lichen Planus (OLP) should be referred to the appropriate specialists to rule out involvement of other mucous membranes and track it early to diagnose a possible malignant transformation. The efficient management of VVGS involves a multidisciplinary approach involving gynecologists, dermatologists and stomatologists.