10.22376/ijpbs.2019.10.1.p1-12 Volume 7 Issue 4 2016 (October - December) Neuronal migration defects are rare causes of seizure disorder and developmental problems. Schizencephaly, the most severe form is an extremely rare entity. Presentation and outcome of Schizencephaly are variable, but it typically presents with hemiparesis, seizures and developmental deficit. Its prevalence in India is 1.5per one lakh population. Schizencephaly is a congenital condition, characterized by cerebrospinal fluid-filled clefts that extend from the pia surface of the cerebral hemisphere to ependymal surface of the ventricle of the brain. Magnetic Resonance Imaging is the modality of choice for its diagnosis. We present a female preterm neonate of 32 weeks gestation 1.35kgs born to non consanguinous parents had reduced tone of the right upper and lower limbs. Ultrasonogram of Cranium showed prominent left lateral ventricle. MRI brain presented with left open lip Schizencephaly. P. MAHARAJA AND P. PARAMANANTHAM Schizencephaly, Brain malformation, Magnetic resonance imaging. 954-956