XY Female with Complete Androgen Insensitivity Syndrome with Bilateral Inguinal Hernia

Abstract

Complete androgen insensitivity syndrome (CAIS) is an X-linked recessive rare disorder in which the individual is phenotypically female and genotypically male; a male pseudohermaphrodite. CAIS is suspected when the individual is evaluated for primary amenorrhea, infertility or when unilateral/bilateral inguinal hernia is diagnosed in girls. We report the case of a 30-year-old, married lady presented to Gynecology OPD with complaints of swelling in the groin, on both the sides since 4 months. She was investigated and all her blood tests were of male range and in accordance with CAIS. Bilateral gonadectomy with herniorraphy was done and the patient was discharged on estrogen replacement therapy.