Schizencephaly is an anomaly of the subtotal brain development, which occurs as the presence of a cleft lined with grey matter extending from subarachnoid space to the ventricles. It may be manifested by psychomotor retardation, paresis or partial seizures and drug-resistant convulsions. The clinical expression of schizencephaly depends on the bilaterality of the slit, its size and its seat. The diagnostic strategy of schizencephaly in the ante- and postnatal period has been revolutionised by MRI imaging, the only technique able to provide an accurate and complete lesional assessment, particularly in type I. We report the case of a 34-year-old pregnant woman at the 25th weeks of amenorrhea, who presented a super-refractory epileptic-status due to a right schizencephaly. The diagnosis of eclampsia was excluded. This case report is very particular cause of the late appearance of epileptic seizures in this pregnant woman who has never done so.