Congenital glioblastoma multiforme is relatively rare accounting for 2-9% of all congenital brain tumors. We deseribe a case of congenital glioblastoma multiforme which occurred in the lateral ventricle. T1-weighted images revealed high signal intensity, with areas of internal low signal intensity, while T2-weighted images showed low signal intensity with focal internal high-signal portions. Post-contrast T1-weighted images depicted a lateral ventricular mass which extended to adjacent brain parenchyme and had a serpentine signal void representing internal vessel.