Complete Androgen Insensitivity Syndrome: Revisiting a Rarity—A Case Report

Complete androgen insensitivity syndrome is a rare disorder. Primary amenorrhea beyond 14 years of age without, and 16 years along with developed secondary sexual characters should raise suspicion of this entity as a cause. Inguinal examination may confirm presence of inguinal gonads. The absence of female internal genitalia on imaging, absence of pubic/axillary hair, and chromosomal analysis clinch the diagnosis. Surgical excision of gonads to prevent malignancy, estrogen supplementation, and vaginoplasty along with psychological counselling remain the mainstay of treatment.