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Objective: To add a new case of primary vulvar lymphangioma circumscriptum to the literature and to review the current literature.

Materials and Methods: We searched the PubMed/MEDLINE databases for previous case reports using the key words "vulvar lymphangioma circumscriptum" and "primary vulvar lymphangioma circumscriptum."

Results: We found 21 cases of primary vulvar lymphangioma circumscriptum reported until 2013. Mean age was 32 years (range = 3-76 y), and 62 % of the cases had been treated surgically.

Conclusions: The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy.

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