Congenital adrenal hyperplasia (CAH) is the result of an autosomal recessive disorder.It affects one of the step required for the synthesis of cortisol from cholesterol by the adrenal cortex. 11-hydroxylase deficiency accounts for 5- 8% of all cases of CAH with an incidence estimated at 1 in 100,000 - 200,000 newborns [1]. We present a 2.5-year-old girl with CAH due to 11- hydroxylase deficiency who presented for surgical treatment of cliteromegaly with hypoplastic lower third of vagina for clitoroplasty with stage 1 vaginal reconstructions. The perioperative care of patients with CAH should address issues related to the deficient and excessive production of specific corticosteroids and mineralocorticoids by the adrenal cortex. We will discuss the perioperative implications of the disorder.