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We report the clinical and neuroimaging features of 4 cases with schizencephaly. Case 1 had bilateral schizencephaly with open-lip on the right and closed-lip on the left. Case 2 had unilateral schizencephaly with closed-lip on the left and subcortical heterotopia on the right. Case 3 had unilateral schizencephaly with closed-lip on the left. Case 4 had bilateral closed-lip schizencephaly. Although all cases except for Case 3 had bilateral lesions, neurodevelopmental outcome was generally good; Case 1 and 3 had mild hemiparesis. All patient have epilepsy which are well-controlled with antiepileptic drugs. Thus, the clinical presentation of schizencephaly, even if bilateral lesions, are quite variable.