Magnetic resonance imaging (MRI) and magnetic resonance spectroscopic (MRS) findings in 3-methylcrotonylglycinuria presenting with acute metabolic decompensation in a previously healthy 7-year old female are described. The patient was hospitalized with fever, irritability, gastrointestinal problems, drowsiness, signs of upper motor neuron deficit, and rapidly progressive respiratory distress requiring assisted ventilation. Laboratory workup showed severe metabolic acidosis, and the diagnosis of 3-methylcrotonylglycinuria was established by the mass spectrometry analysis of urine sample. Although initial CT imaging workup was found to be gross normal, subsequent MRI of the brain in the early chronic stage of the disease showed symmetrical ill-defined signal abnormalities within medulla oblongata, pons, inferior cerebellar peduncles, and periventricular white matter in cerebral hemispheres. Diffusion-weighted images were unremarkable. Single-voxel proton MRS showed elevated levels of lactate, branched-chain amino acids, as well as glutamine and glutamate. To the best of our knowledge, this is the first reported case of late onset 3-methylcrotonylglycinuria with complete MRI and MRS workup in the early chronic phase after metabolic crisis.