We presented MR (magnetic resonance) images findings of the brain in nonketotic hyperglycinemia (NKH) from 2 patients of neonatal onset. MR findings in our cases of NKH are compatible with the pathological alterations described in the literature; MR images revealed delayed myelination of the cerebral white matter and hypoplasia of the corps callosum. The latter finding particularly seemed to be relatively characteristic to NKH. MR examination is the imaging modality of choice for this disease because it can demonstrate the degree of myelination, and development of the corpus callosum particularly on sagittal section.