Glucose is a metabolic fuel in the body especially for the brain. Glucose homeostasis is based on storage of carbohydrates, proteins and fats. Interconversion is regulated by several hormones and enzymes. Hypoglycemia in IEM is mainly due to inadequate stores, or immature enzymes, abnormalities in insulin production, disorders of carbohydrate, protein and fat metabolism. Material: A total of 40 neonates, Infants and children presenting with hypoglycemia of <50 mg/dl were investigated for inborn errors of metabolism. Results: Two of the neonates were premature at 28 weeks gestation, Beta ketothiolase deficiency in two neonates, CPT1 in one infant, 2 of MCAD deficiency, 2 of Glycogen storage disease (GSD 1 one case, GSD3 one case). 17.5% of children with hypoglycemia had an inborn error.