Unusual computed tomographic findings were observed in two infants with similar clinical pictures including delay in developmental milestones, seizures, decerebrated posture, and microcephaly. Computed tomography revealed small brain with widened subarachnoid space, smooth surface of the brain, uniformly enlarged ventricles, wide sylvian cisterns, and lack of insular opercularization. These findings coincide with pathologic features of lissencephaly (agyria), a rare congenital anomaly of the brain due to interruption of neuronal migration.