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. A 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. What is Known: • Lipschütz's acute vulvar ulceration is a non-sexually acquired condition, which is characterized by a sudden onset of a few necrotic and painful genital ulcers. • The condition tends to resolve spontaneously and is usually triggered by an infection. What is New: • The condition mainly affects subjects ≤ 20 years of age, tends to resolve within 3 weeks, and is usually temporarily associated with a flu-like illness or an infectious mononucleosis syndrome. • Systemic corticosteroids do not reduce disease duration.