Miller-Dieker syndrome is an autosomal dominant congenital disorder characterized by lissencephaly (smooth brain), which is resulted from incomplete neuronal migration. The disorder arises from the deletion of part of the small arm of chromosome 17p (which includes both the LIS1 and 14-3-3 epsilon gene), leading to partial monosomy. Here we present a brief introduction to the common knowledge of Miller-Dieker syndrome.