Isovaleric acidemia is an autosomal recessive inborn error of
metabolism caused by deficiency of mitochondrial enzyme isovaleryl-CoA dehydrogenase (IVD) resulting in the accumulation of derivatives of isovaleryl- CoA that can cause significant morbidity and mortality. Acute metabolic encephalopathy can be subtle in the neonatal period and can’t be always recognized because many presentation can occur. We present a case of acute metabolic encephalopathy in a 7-day-old boy who came to the Emergency Department through referral with the diagnosis of suspected of neonatal sepsis. He was poorly feeding since 2 days before admission and had a history of seizure. The baby was very lethargic and inactive. There was a peculiar body odor like “sweaty feet”. His clinical and laboratory examination showed the following abnormalities: severe dehydration, positive pathologic reflexes, acute kidney injury stage failure, metabolic acidosis, hypernatremia, hyperkalemia, hypercalcemia and hyperglycemia, high anion gap, hyperammonemia and normal limit of lactate. It was suggestive for Isovaleric Acidemia so before the tandem mass spectrometry result revealed the diagnosis, we managed this patients by fluid rescucitation, electrolyte correction, protein restriction and carnitin supplementation. We did not give glycine since its not available in Indonesia. After a month of hospitalization, his clinical condition had improved, and was followed as an outpatient clinic patient.