Primary adrenocortical insufficiency due to congenital hypoplasia or atrophy of the glands has been estimated to have a frequency of 1 in 15000 births1 .CAH includes a group of autosomal , recessively inherited diseases with impaired cortisol synthesis. Five different enzymatic conversions in sequence are essential for the production of cortisol from cholesterol .As patients now survive into adulthood , adult health care providers must also be familiar with this condition. Here we report a 55 days old female child with chief complaints of vomiting since 1 month , not gaining weight and hypopigmented patches over buttocks and thighs .