Background: Acquired lymphangioma circumscriptum is among the rarest and most benign lymphatic system conditions. The etiology of lymphangioma circumscriptum is unclear Case presentation: a case was presented of a 42-year-old Para 4 woman diagnosed to have acquired lymphangioma circumscriptum following presentation with vulvar swelling for which a simple partial vulvectomy was done Conclusions: The clinical presentation of a patient with acquired vulvar lymphangioma circumscriptum may resemble genital warts and malignancies. For a patient with vulvar swelling, vulvar lymphangioma should be called a differential diagnosis. Simple partial vulvectomy is one option for management if Sclerotherapy and vaporization with CO2 are not available.

Keywords: Vulvar swelling, acquired vulvar lymphangioma circumscriptum, simple partial vulvectomy, case report