An encephalocele is a developmental disorder of neural tube closure, where as schizencephaly is a developmental disorder of neuronal migration. There are only few case reports suggesting association of schizencephaly with meningoencephalocele. We report a case of 6-year-old child who was brought by her parent with complaints of progressive swelling over her vertex since birth, left sided hemiparesis and intermittent generalized tonic clonic convulsion for last 3 years. Clinical examination, computed tomography (CT) and magnetic resonance imaging (MRI) of brain revealed a large calvarial defect with meningoencephalocele and associated schizencephaly. Initially, ventriculoperitoneal (VP) shunt was carried out, followed by successful repairing of the defect. This case is interesting for late presentation, management difficulties and associated anomalies "schizencephaly", which is a rare developmental disorder.
Keywords: Microcephaly; schizencephaly; vertex meningoencephalocele.