Maple syrup urine disease (MSUD) is caused by a genetic defect of branched-chain amino acids, which include leucine, isoleucine and valine. We report diffusion-weighted imaging (DWI) findings in a newborn child with MSUD who presented with acute metabolic encephalopathic crisis. DWI (b = 1,000 s/mm(2)) showed high signal localized within the myelinated white matter (WM) areas including the cerebellar white matter, pons, bulbus, cerebral peduncles, lentiform nucleus, posterior limbs of the internal capsules, corona radiata and bilateral perirolandic cortex. The apparent diffusion coefficient values of these regions were markedly low in the affected areas. The presence of these findings was considered cytotoxic or intramyelinic edema evidenced by restricted water diffusion. In conclusion, our findings suggest that during the acute phase and early encephalopathic crisis stage of MSUD, DWI can demonstrate the involvement of myelinated WM in newborns.