Abstract

Background: Hermaphroditism or intersex is a condition in which the individual’s genitalia may be ambiguous. It is possible for the ovarian and testicular tissues to be separated or to fuse together to form an ovotestis, which is extremely rare in humans.
Case Presentation: A 38-year-old Saudi woman came to the clinic complaining of primary infertility for the past 19 years. She was married to a 40-year-old male. The wife complained of masses in her labia that are sometimes painful during sex. On examining the labia on both sides, a small mobile mass was tender to strong touch toward the lower part of the labia and can be reduced intraabdominally through the inguinal ring. This raised the suspicion of being a hermaphrodite. On karyotyping, the patient was found to have XY chromosome. Bilateral orchidectomy was conducted. The patient was discharged on the third day, well, and was seen after 6 weeks and 12 weeks in good health.
Conclusion: Androgen insensitivity syndrome is a rare disease that should be acknowledged by doctors when diagnosing patients with ambiguous genitalia or later in life with infertility. Adult patients with AIS might be challenging to manage, but family support is one of the pillars of management.

Key words: Androgen insensitivity syndrome, infertility, orchidectomy, labial mass, hermaphrodite, karyotype, case report