Tehran University of Medical Sciences Acta Medica Iranica 0044-6025 42 6 2004 12 15 458 460 H. Sharami F. Ghaemmaghami F. Yarandi F. Milani N. Alizadeh 2015 09 28 Lymphangioma circumscriptum is a benign lymphatic malformation which is localized to the skin and subcutaneous tissues. It consists of dilated lymph channels lined by normal endothelium.Primary vulvar involvement is very rare and only 28 cases have been reported till 2002. Here we report a case in an 18 years old girl who was referred to our gynecologic clinic with symptoms of pain,swelling and erythema of both labia majors of 5 years duration. She had taken various drugs but all had failed and the lesion was exterminated only after wide local excision surgery. Histological examination revealed multiple dilated vascular channels with an inflammatory infiltrate in papillary dermis and diagnosis of vulvar lymphangioma circumscriptum was made. After ten months follow up, there was no evidence of recurrence. Wide local excision may be the best treatment for extensive vulvar lymphangioma circumscriptum. https://acta.tums.ac.ir/index.php/acta/article/view/2773 https://acta.tums.ac.ir/index.php/acta/article/download/2773/2755