Oxidative stress has been demonstrated to be an underlying patho-physiologic process in various inborn errors of metabolism. Metabolic profiling of oxidative stress may provide a nonspecific measure of disease activity that may further enable physicians to monitor disease. In this study, we try to find the correlation between the oxidative stress and inborn. Quantifying oxidative stress offers a unique perspective to IEM. Certain measures may provide a means of addressing mitochondrial function in IEM and aid in the development of therapeutic targets and clinical monitoring in this diverse set of disorders.