Psychotic symptoms have been reported in association with a wide array of brain abnormalities. Few published reports have examined the association between schizencephaly and psychiatric illness. Originally defined by Wilmarth and later by Yakolev and Wadsworth – Schizencephaly is an uncommon congenital disorder of cerebral cortical development, defined as a grey matter-lined cleft extending from the pial surface to the ventricle. The nosology is based on neuroradiologic findings and confirmed by neuropathology when available. The Clinical presentation and neurodevelopmental outcomes of the disorder vary and are usually related to the extent/areas of the brain involved. In this article we review the medical literature around Schizencephaly paying particular attention to the pathophysiology, etiology and diagnosis of such patients. We then present a case of Schizencephaly and first episode psychosis in a 16-year-old adolescent who was admitted to our inpatient psychiatric service. Lastly, we present the findings of a systematic review from PubMed whereby we summarize 10 cases of Schizencephaly with associated psychiatric symptoms.