Inguinal hernia is the most common type of groin hernia in females. In comparison, complete androgen insensitivity syndrome (CAIS) is a rare genetic disease that can present with an inguinal swelling containing testis in a female. We report a case of a 42-year-old phenotypic female who presented with swelling in the right inguinal region and primary amenorrhea. The swelling showed impulse on coughing, and working diagnosis of incarcerated hernia was made. Investigations revealed the absence of uterus, cervix, and fallopian tube with presence of right ovary in right inguinal canal and left ovary in intra-abdominal region. Chromosome analysis revealed XY karyotype, thereby confirming the diagnosis of CAIS. On exploration, firm testis-like structure was detected in the right inguinal canal, and gonadectomy with herniotomy was done. However, opposite side gonadectomy was refused by the patient. Histopathologically, excised mass showed atrophic testis with sertoli cell adenoma.
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