Schizencephaly is a central nervous system (CNS) developmental disorder characterized by abnormal cleft extending from the lateral ventricles to the cerebral cortex. Clinically, it occurs as trans -mantle, closed lip and open lip types which may be unilateral or bilateral. The exact cause of schizencephaly is not known but genetic disorders, exposure to teratogens, viral infections and maternal age are implicated. We present a case of bilateral open lip schizencephaly with some degrees of neurological disorders caused by increased intra-cranial pressure (ICP) due to ventriculomegaly. We applied ventriculo-peritoneal shunt (V–P shunt) to the patient with considerable improvement after post-operative follow-up.