Introduction: Lymphangiomas are benign tumors/malformations, characterized by proliferation of the lymphatic vessels. They may arise anywhere, although the most common localizations are the head-neck region and the axilla. To date, only 21 cases of lymphangioma of the ovary in a 60-year literature survey have been reported.
Case report: A 16-year-old female patient with long standing abdominal distension had 40 cm × 15 cm × 29 cm ovarian lymphangioma.
Conclusions: Our case highlights that lymphangiomas can occur in the adolescent population and should be added to the differential diagnosis of ovarian masses in this age group.
Keywords: Ovarian lymphangioma; abdominal distension; endothelial marker D-240; lymphatic system; pediatric age.