Lymphangioma circumscriptum is a rare, usually benign, dermatological condition that can be acquired or congenital. Acquired lymphangioma circumscriptum is often found in the axillae, genitals, buttocks, or inguinal regions. It can be associated with malignancy, radiation, or surgery. It is often misdiagnosed due to its variable appearance and similar features to other dermatological conditions.

This case is an 11 year old with past medical history of anaplastic large cell lymphoma who presented to surgery for scheduled IUD insertion. At time of surgery, a lower left vulvar lesion was observed with aytpical features concerning for possible melanoma. The lesion was excised and sent to pathology, where it was diagnosed as lymphangioma circumscriptum.

Lymphangioma circumscriptum is rare and often misdiagnosed. Our aim in presenting this case study is to further expand the description of lymphangioma circumscriptum and its consideration in an unknown vulvar skin lesion.