Lipschutz ulcers refer to the ulceration of vulva or lower vagina of non-venereal origin that usually presents in young women, especially virgins. We report a case of acute ulceration of vulva in an adolescent patient, who was initially diagnosed as suspected Behcet’s disease, and was finally diagnosed as Lipschutz ulcer. A 14-year-old female visited a gynecology clinic with a single acute ulceration of vulva. Her past history showed frequent ulceration of oral cavity, and she was sent to our dermatology clinic for a suspected Behcet’s disease. She was presented with fever, malaise and mild pharyngitis before development of the ulcer, but had no history of sexual contacts. We underwent serologic tests to rule out infectious etiologies. The bacterial swab showed no growth of pathologic flora, and the serologic labs for Epstein-Barr virus showed positive for EBV antibodies to IgG and IgM. When direct cytotoxic infections and specific systemic diseases for acute genital ulcer have been excluded, the patient meets the criteria for Lipschutz ulcers. Because of high incidence of Behcet’s disease in the East Asia, it is easy to misdiagnose as Behcet’s disease when patients present with recurrent aphthous stomatitis history with acute ulceration of vulva. Therefore, main causes of genital ulcer in young females have been excluded, and there is no history of sexual contact, Lipschutz ulcer should be included in the differential diagnosis.