Androgen insensitivity syndrome in an Indian adult

In androgen insensitivity syndrome (AIS), the patients are phenotypically females with male karyotype. Affected persons present at puberty with primary amenorrhea with adequate breast development and normal external genitalia. The gonads may be found in lower abdomen, inguinal regions, or sometimes in the labia majora. Rarely, the testes may undergo malignant transformation. So, gonadectomy is indicated in these patients after they attain puberty. Here, we present a case of AIS with primary amenorrhea and bilateral inguinal swelling.