Abstract: - Congenital Adrenal Hyperplasia (CAH) is an autosomal recessive disorder that is associated with deficiencies in 21, 11, or 17-hydroxylase. More than 90% of congenital adrenal hyperplasia is caused by 21- hydroxylase deficiency which is found in 1:10,000 to 1:15,000 live births, characterized by impaired glucocorticoid & mineralocorticoid synthesis.

We are presenting a one year old female child, weighing 12 kg with classical 21- hydroxylase deficiency (salt wasting type) posted for clitoroplasty, diagnosed at the age of 3 months and started on tab. hydrocortisone and tab. fludrocortisone with frequent monitoring of parameters such as serum 17-hydroxy progesterone andandrostenedione levels, to assess the therapeutic control. Preoperatively, after optimization patient was taken up for surgery under general anaesthesia with perioperative steroid therapy. The surgery went uneventful. After surgery patient was extubated on table and monitored in the postoperative recovery room.

The usual presentation is failure to thrive, hyperkalemia, hyponatremia and features ofhyperandrogenism. Anaesthetic considerations in such cases include electrolyte disturbances, acute adrenal crisis in untreated patients, complications due to steroid therapy like difficult intravenous catheter placement, difficult airway due to obesity, hypertension, hyperglycaemia, hypovolemia, positioning considerations due to osteoporosis& even prolong the effect of the muscle relaxants.

Such cases can be managed successfully after understanding the pathophysiology of disease with proper history, preoperative optimization, perioperative steroid supplementation.