A patient with Miller-Dieker-Syndrome, which is associated with lissencephaly, was examined by Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). While CT demonstrated the main features of lissencephaly, MRI detected disturbed myelination and cell migration in the cerebral hemispheres and a normal appearance of the cerebellum. MRI provides more accurate information of the pathomorphologic changes of lissencephaly and is thus superior to CT.