RÉSUMÉ
This is a case of a 23 years-old single, nulligravid with primary amenorrhea, sexual infantilism, tall stature who presented with abdominal enlargement. Ultrasound showed a small uterus, thin tall stature who presented with abdominal enlargement. Ultrasound showed a small uterus, thin endometrium, bilateral adnexal solid masses. Extensive diagnostic work-up for the etiology of the priamry amenorrhea led to the diagnosis of Swyer syndrome. Karyotyping showed 46, XY. On laparotomy, both gonads were solid with tumor implants on the omentum, mesentery and serosa of the small uterus. Total hysteroctomy with bilateral salpingo-gonadectomy, infracolic omentectomy, tumor debulking and biopsy of implants were done. Histopathologic report showed yolf sac (endodermal sinus tumor), both gonads and aforementioned implants. The risk of neoplasia in such cases is discussed.