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Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder. It is caused by a deficiency of the branched chain α-ketoacid dehydrogenase enzyme complex, leading to accumulation of the branched chain amino acids (leucine, isoleucine, and valine) and their toxic byproducts (ketoacids) in the blood and urine. Imaging is characterestized by MSUD oedema affecting the myelinated white matter. We present a neonatal case with classic type of MSUD with radiological findings confirmed byfatcat:2kpww22s7zgffjdse2xgckpjju