Schizencephaly is a rare malformation of the central nervous system, a congenital disorder of cerebral cortical development resulting in the formation of abnormal unilateral or bilateral clefts in the cerebral hemispheres that extends from the pial surface to the ventricle. It often manifests with partial seizures, mental retardation and hemiparesis.

To illustrate a rare case of association between psychosis and schizencephaly and the implication of this association for understanding the biology of the psychosis.

A literature search was performed on PubMed database using the key words schizencephaly, psychosis, brain diseases and retrieved papers were selected according to their relevance. The patient clinical record was reviewed.

The authors report a case of a 59-year-old male admitted into a psychiatric hospital with insomnia, disorganized behavior probably secondary to auditory hallucinations and mystic delusions. He also reported epilepsy and strabismus in his right eye since his childhood and right facial paresis. A head CT scan revealed a left deep cortico-ventricular parieto-occipital communication corresponding to schizencephaly.

Considering the theory that schizophrenia is associated with abnormal brain development, this case report may provide an example of a neurodevelopment abnormality that manifests as psychosis.