Disorders of sex development (DSD) are medical conditions in which the development of chromosomal, gonadal or anatomic sex varies from normal and may be incongruent with each other. Swyer syndrome is a condition caused by pure gonadal dysgenesis 46,XY, which followed by inadequate anti-mullerian (AMH) production results in maintenance and further development of mullerian duct into uterus. Therefore, many patients who suffer from this condition initially come with chief complaint of primary amenorrhea with insufficient development of secondary sexual characteristics. Patient’s gender orientation commonly is a female, since her brain was not sufficiently exposed with androgen hormone. Management of DSD patients should be based on patient-centered approach. Therefore, overall management of DSD patients should follow according to patient’s perception regarding to her gender orientation. Herein, we reported a case of Swyer syndrome in female aged 29 years with chief complain primary amenorrhea. Chromosomal analysis was 46,XY (20 mataphase), FSH level was 31.5miu/ml, LH 10.8miu/ml, estradiol (E2) <5pg/ml, testosteron total (T) <0.0025ng/ml. Medical management for this patient has the purpose to drive her feminization process in order to improve her physical appearance. However, since there is a great tisk for having gonadal tumor development from intra-abdominal dysgenetic gonad with Y chromosome, this patient has been strongly suggested to have bilateral gonadectomy.