Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 2 4 2018 05 14 94 97 EN Shahram Shabaninia Department of Urology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. Seyed Reza Yahyazadeh Department of Urology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. Amin Azadfar Department of Urology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. Mahrouz Amirheydari Department of Urology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. 2017 09 29 2018 01 10 Swyer syndrome is a very rare cause of primary amenorrhea. Affected individuals have an XY karyotype but their external and internal genitalia are of the female type. The gonads are usually replaced by fibrous streaks. Early diagnosis is vital because of the significant risk of germ cell tumor, and bilateral gonadectomy should be performed. Laparoscopy provides a minimally invasive approach for the management of these cases. These patients can have a normal sexual intercourse and they need hormone replacement therapy for development of breast and prevention of osteoporosis. They can conceive through oocyte donation and artificial reproductive techniques.     https://crcp.tums.ac.ir/index.php/crcp/article/view/135 https://crcp.tums.ac.ir/index.php/crcp/article/download/135/89