Introduction: Schizencephaly is a rare congenital disorder characterized by the presence of a cleft that extends from the cerebral cortex to the lateral ventricles. The prevalence of schizencephaly is estimated to be 1.48 per 100,000 births. Currently, it is divided into three types: trans-mantle, closed-lip and open-lip.

Case Description: A newborn female was admitted to the neonatology intensive care unit immediately after birth due to multiple birth defects including frontal bossing, low-set ears and digits malformation. This was accompanied by neurological manifestations; mainly generalized hypotonicity. Thorough investigations revealed a large bilateral open lip schizencephaly.

Discussion: Congenital malformations are present in 2-3% of all births globally. It ranges from mild cosmetically repaired defects to life threatening conditions. Open lip schizencephaly is an extremely rare neurologic malformation that has multiple presentations and prognosis depending on the extent of brain tissue affected.

Conclusion: Congenital malformations are related to genetic and environmental factors. This is why a multidisciplinary approach is required in most cases.

Keywords: Open lip Schizencephaly; Malformation; Neonate