Incidental Inguinal Tubercular Lymphadenitis in a female of Androgen Insensivity Syndrome- A rarest of rare presentation

Androgen insensitivity syndrome (AIS) is a rare X-linked recessive disorder occurring in phenotypic women with male genotype (46, XY) resulting due to mutation the X chromosome. On other hand, tuberculosis (TB) chronic granulomatous infection caused by Mycobacterium tuberculosis, an acid-fast bacillus. We report case of 22-year-old unmarried female presenting bilateral inguinal pain and primary amenorrhea, which, on comprehensive evaluation, revealed complete external genitalia intra-abdominal testes. The karyotype was 46 XY, hence diagnosis AIS made. One ‘probable testes’ removed surgically lymph node tuberculous lymphadenitis. Isolated lymphadenopathy, itself, rare. Co-occurrence Complete lymphadenopathy highly unusual association that has not been documented literature reviewed.