Maple Syrup Urinary Disease (MSUD) is an autosomal regressive irregularity featured by a maple syrup smell in the urine, resulting from abnormalities in leucine, isoleucine and valine metabolism. The incidence of this disorder in our country is 1/200,000. The major clinical characteristics submitted by MSUD the sicks include ketoacidosis, growth moreover developmental delay, undernourishment, apnea, ataxia, seizures, coma, psychomotor postpone, hyperactivity moreover mental retardation. There are five kinds of this disease: classical, episodic, medium, thiamine sensitive, in addition E3 deficiency. Classic MSUD, which is usually encountered in newborn babies, can result in coma due to malnutrition and, if not treated, death. The beginning of signs varies base on the quantity of protein intake. Most patients who are cured within a few days of the beginning of signs are out of danger moreover might not improve any neurological harm. Afterwards the acute crisis assaults are resolved, longtime nutritional treatment with a focus on restricting branched chain amino acids (BCAAs) should be initiated and go aheaded in perpetuity. Latest studies have confirmed that if nutritional therapy is started early, brain damage that may occur is minimized. Any imbalance between free radicals moreover antioxidants give rise to oxidative damage to proteins, fats, nucleic acids and carbohydrates. Such a situation is called oxidative stress. In today's conditions, the research done and conducted on oxidative stress has attracted a lot of attention from scientists. Free radicals, which are molecules with free electrons on atomic orbitals, lead to the emergence of oxidative stress. In this research, it was targeted to underline the significance of the relationship between maple syrup urine disease and oxidative stress.