Background- Schizencephaly is a rare disorder of neuronal migration which is characterized by clefts in cerebral mantle which communicate between subarachnoid spaces laterally and ventricular system medially. rnCase report- An extremely rare case of bilateral giant open lip schizencephaly with massive subdural effusion is presented here in with relevant literature.rnConclusion- There are no guidelines for the treatment of schizencephaly; overall prognosis of schizencephaly remains guarded and depends upon the amount of preserved cortical mantle and associated anomalies.