Schizencephaly is a regional disturbance of cerebral hemisphere formation occurring at 3-5 months gestation; neonatal presentation is uncommon. Three neonates with schizencephaly were evaluated with cranial ultrasonography (US), unenhanced computed tomography (CT), and magnetic resonance imaging (MRI) examinations. Common findings in US, CT, and MRI include parasylvian and midline clefts, size asymmetries of the basal ganglia and thalamus, cerebral parenchymal volume loss, ventriculomegaly, ventricular diverticula, and absence of the septum pellucidum. MRI and CT were superior to US in detecting calcification, gyral and sulcal abnormalities, and parasylvian clefts. MRI alone demonstrated homolateral absence of the sylvian vasculature, small medullary pyramids, low position of the fornix, and the thinning of the corpus callosum. Although US appears adequate as a screening test, MRI best defines the precise pathoanatomic findings of neonatal schizencephaly and allows for the prediction of neurologic outcomes in affected newborns.