Abstract

Complete androgen insensitivity syndrome (CAIS) is a rare androgen receptor function disorder where phenotypic female has a male genotype. They mostly present as bilateral inguinal hernias containing testes. Uterus and cervix are absent. The authors present a case of CAIS in a 22-year-old female where the presence of testes, prostatic tissue and seminal vesicles was confirmed by ultrasonography, hormonal analysis, operative findings and histopathological study. She was second of the two sisters and her elder sister was also diagnosed with CAIS. High levels of testosterone seen in CAIS can stimulate Wolffian duct development/differentiation. Gonadectomy is advisable in such patients to avoid future neoplastic changes.

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