A 12 year old boy was brought by his parents with inability to speak and floppiness since childhood.
The MRI of the brain shows partial corpus callosal dysgenesis with ventriculomegaly. The posterior fossa shows a batwing appearance of the fourth ventricle, vermian hypoplasia and prominent, thickened and elongated superior cerebellar peduncles with a deep interpeduncular fissure giving the characteristic ‘molar tooth’ appearance.