Schizencephaly (from Greek skhizein 'to split' and enkephalos 'brain')[1][2] is a rare birth defect of the brain, characterized by abnormal clefts lined with grey matter that form the ependyma of the cerebral ventricles to the pia mater.

Common clinical features of this malformation include epilepsy, motor deficits, and psychomotor retardation.

[4] In schizencephaly, the neurons border the edge of the cleft, implying a very early disruption of the usual grey matter migration during embryogenesis.

The prognosis for individuals with schizencephaly varies depending on the size of the clefts and the degree of neurological deficit.

Because having a sibling with schizencephaly has been statistically shown to increase risk of the disorder, it is possible that there is a heritable genetic component to the disease.