It is an autosomal recessive disorder caused by deficiency of Glutaryl CoA dehydrogenase. Patients may develop normally upto 2 years of age as was seen in our patient. Macrocephaly may be seen. After a minor infection, symptoms of hypotonia, choreoathetosis, seizures, dystonia may occur. Recovery from 1st attack is slow and some residual neurologic abnormality may persist. Similar episodes may recur during an intercurrent infection and metabolic decompensation with vomiting, ketosis, seizures and even death may occur. Some patients may develop rigidity and dystonia over period of years through intellect may remain normal. Investigations may reveal metabolic acidosis, ketosis, hypoglycemia, hyperammonemia or elevated serum transansinases in some patients. Our patient had elevated transaminases. 3-Hydroxy glutaric acid is elevated in urine and high concentrations of glutaric acid may be seen in urine, blood or CSF. Plasma amino acids are normal. Treatment consists of low protein diet, high doses of riboflavin and carnitine. Prenatal diagnosis can be done by demonstrating increased concentration of glutaric acid in amniotic fluid.