Abstract
Ovarian Sertoli-Leydig cell tumors (SLCT) are extremely rare malignant tumors deriving from the sex cord stroma. An abdominal mass and a virilization syndrome dominate the clinical symptoms. This particular tumor poses diagnostic and therapeutic problems. Prognosis depends on staging (the International Federation of Gynecology and Obstetrics (FIGO)/tumor, node, metastasis (TNM)) and differentiation. The treatment is surgical, combined with adjuvant chemotherapy in certain cases. We report the case of a three-year-old girl admitted to our department for signs of virilization with an abdominal mass. The literature does not contain any reports of a younger case. Ovarian SLCTs should be considered in every girl presenting with signs of virilization and a lower abdominal mass. The prognosis and management depend on the results of the histological analysis and extension evaluation in order to define therapeutic management.