We present conventional magnetic resonance (MR) imaging with diffusion-weighted and diffusion-tensor imaging findings in a 10-day-old neonate with maple syrup urine disease (MSUD). On conventional MR imaging, signal abnormalities were noted in the affected white matter of cerebellum, dorsal brainstem, thalami, posterior limbs of internal capsules, and the corona radiata. These regions showed marked hyperintensity on diffusion-weighted images with decreased apparent diffusion coefficient values (average 68% reduction). Diffusion-tensor imaging showed decreased anisotropy (average 57% reduction) in the corresponding areas. Both diffusion-weighted and diffusion-tensor imaging are valuable in the diagnosis and understanding of the pathogenesis of MSUD, with findings that suggest cytotoxic edema and damaged oligodendro-axonal units within the affected white matter.