In previously healthy subjects, vulvar ulcers are mostly caused by sexually transmitted microorganisms. Lipschütz's acute vulvar ulceration, first reported in 1912, is a non-sexually acquired condition characterized by sudden onset of a few genital ulcers. We systematically review presentation, underlying causes and disease duration of Lipschütz's ulceration.Comprehensive source of Excerpta Medica, National Library of Medicine and Web of Science databases was performed. Reports including cases of apparently previously healthy females affected by Lipschütz's ulceration were selected a predefined database was used to extract data on demographics, history, clinical and microbiological findings, and treatment.The search disclosed 158 cases. Almost 90% of cases were ≤20 years of age and sexually inactive. Lesions were usually one to about three, painful, ≥10 mm large, well-delimited, with a fibrinous and necrotic center and a symmetric distribution. Voiding disorders and enlarged inguinal lymph nodes were observed in a large subset of cases. Canker sores were noted in 10% of patients. Lipschütz's vulvar ulceration occurred concomitantly with an infectious disease in 139 cases. Infectious mononucleosis syndrome (N=40) was the most frequently detected well-defined infection, followed by mycoplasma species infections (N=11). The disease resolved after ≤3 weeks. CONCLUSIONS: Lipschütz's ulceration mainly affects both sexually inactive and, less frequently, sexually active subjects ≤20 years of age, presents with ≤3 vulvar ulcers, resolves without recurrences within 3 weeks and is temporarily associated with an infection, most frequently a flu-like illness or an infectious mononucleosis syndrome.