Miller-Dieter syndrome consists of severe type I lissencephaly, abnormal facial appearance, and sometimes other birth defects. Lissencephaly is a brain malformation manifested by a smooth cerebral surface, thickened cortical mantle, and microscopic evidence of incomplete neuronal migration. It comprises the agyria-pachygyria spectrum of malformation, thus excluding polymicrogyria and other cortical dysplasia. Type I lissencephaly results from abnormal migration between about 10 and 14 weeks gestaion. The brain is often small, and the ventricle is enlarged posteriorly The corpus callosum may be small or absent. The structural pattern of the cerebral hemispheres and ventricles is distintly immature, reminiscent of fetal brain. The superficial cellular layer resembles an immature cortex, with some separation into zones similar to layers III, V, and VI of normal cortex, although the cell population is decreased. In 1963 Miller described a malformation syndrome in a brother and sister with postnatal growth deficiency, craniofacial defects, and serious abnormalities of neurologic function. Autopsy at 3 and 4month of age, respectively, revealed lissencephaly. Subsequently, Dieker reported four additional patients with this disorder and referred to it as the 'lissencephaly syndrome'. We have experienced a case with this syndrome. Then we report this rare case with brief review of literature.