Background: Lymphangioma circumscriptum (LC) is a benign dilation of lymph channels localized to the skin and subcutaneous tissues. Primary vulvar involvement is very rare, with only 34 cases reported.
Case: A 17-year-old girl presented with pain and an 18-cm mass of the right labium majus that started 4 years earlier. She underwent wide local excision, and histological examination showed multiple dilated vascular channels in papillary dermis with a diagnosis of vulvar LC.
Conclusion: Vulvar lymphangioma may mimic other vulvar tumors. Excision may be the best treatment of extensive vulvar LC.