Schizencephaly is an uncommon congenital disorder of cerebral cortical development, defined as a gray matter-lined cleft extending from the pial surface to the ventricle. It is a neuronal migration anomaly, caused by insults to migrating neuroblasts during 3rd to 5th gestational months.

Ischemia, germline mutations, intrauterine infections and exposure to drugs have been implicated in its etiology. The outcome relates with the severity of pathology. Unilateral closed lip schizencephaly has the mildest clinical picture and bilateral open lip the most severe. The most prominent manifestations are motor deficits and seizures.

Schizencephaly has been related with psychosis. However, there is paucity of literature exploring this relation. Pubmed search with “Schizencephaly AND Psychotic disorders OR Bipolar Disorders” as Mesh terms resulted in 9 results. Of these four discussed Schizencephaly. Rest five reports were related to other disorders of cortical malformation. We present an interesting case of schizencephaly associated with psychosis and congenital hemiparesis. We also present a review of literature available for this rare association.

This case points towards the role of neurodevelopmental abnormalities in the manifestation of psychosis and bipolar affective disorder. It indicates that presence of neurodevelopmental anomalies may have pathoplastic effects on the presentation of psychosis and may also influence treatment response adversely. A possible mechanism explaining the development of psychosis in schizencephaly is the disruption in intracortical connections. There is also a possibility of underlying ictal phenomenon leading to psychosis. The above case provides support to the neurodevelopmental theory of Schizophrenia.