An X-linked recessive androgen receptor disorder is called complete androgen insensitivity syndrome. It is distinguished by a female XY karyotype phenotype. The external genitalia of the female are normal, but the Mullerian duct derivatives that is, the uterus, cervix and the proximal section of the vagina are absent. However, the intraabdominal, labial, or inguinal testes are present. Up to 5 males out of every 100,000 develop it. Prior to surgical intervention, imaging is crucial for gonad diagnosis and localization.
In this article, we present a case of primary amenorrhea in a teenager who had complete androgen insensitivity syndrome.
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