Intruduction: Testicular feminization syndrome is a disorder with the prevalence of 1 in 20000 to 64000 male births. It,s characteristics are female phenotype, male caryotype (64XY) and normal to increased testosterone and LH levels. In this article a case of testicular feminization is reported. Case: The patient was a 16 years-old woman with primary amenorrhea and without secondary sexual characteristics. The patient had normal female external genitalia, absent axillary and pubic hair, immature breast, and repaired bilateral inguinal hernia with pathology report of immature testicular tissue. FSH and LH levels were elevated. Testosteroe level was normal. Sonography and CT scan of pelvis did not show uterus and adnex. Karyotype was 46XY. Conclusion: It is recommended that due to the risk of neoplasia, the existing testes must be removed. Also hormone therapy must be done for the patient.

Keywords: Testicular feminization syndrome, primary amenorrhea, inguinal hernia

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