Complete androgen insensitivity syndrome: report of a case with solitary pelvic kidney

Complete androgen insensitivity syndrome, commonly known as the testicular feminization syndrome, is characterized by a 46, XY karyotype, bilateral testes, absent or hypoplastic Wolffian ducts, and female-appearing external genitalia with diminished axillary and pubic hair development. Although initial diagnosis in the child is difficult, the syndrome must be suspected after puberty if primary amenorrhea is present. Coexistence of genital defects with urologic abnormalities is expected in these cases because of close embryologic origin. However, unilateral renal agenesis with pelvic ectopia of the contralateral kidney does not seem so common. We report a case of testicular feminization syndrome with a solitary kidney located in bony pelvis on the left side.