Congenital lymphangioma circumscriptum of the vulva

Lymphangioma circumscriptum is a rare benign disorder of unknown etiology affecting lymph vessels in the deep dermal and subcutaneous layers. The commonest sites of presentation are the axilla, shoulders, groins, tongue and oral cavity. Lymphangioma circumscriptum of the vulva is very rare can be either congenital or secondary to lymphadenectomy or pelvic radiation in patients with genital cancer, genital tuberculosis, Crohn's disease, cellulitis, morbid obesity and immobilization. We report the case of congenital lymphangioma circumscriptum of the vulva in an adolescent with congenital lymphedema of the leg.