Congenital adrenal hyperplasia (CAH) is the most common cause of ambiguous
genitalia in newborns. This paper is based upon review of the literature an
d personal experience. We focus upon the surgical anatomy, pre-operative ev
aluation, including imaging, mainly by transabdominal ultrasound, and upon
the goals and the history of surgical reconstruction. The various surgical
techniques are mentioned with a detailed description of our technique used
in 52 patients. The timing and staging of the operation and the implication
s of prenatal therapy are discussed. In conclusion, we believe that infants
with 46XX CAH can undergo one-stage feminizing genitoplasty very early in
life with satisfactory cosmetic and functional results.