A 5-year-old boy with a large V-pattern exotropia is described. He has a background of congenital left hemiparesis associated with schizencephaly. At the time of surgery the lateral recti were found to be abnormally inferiorly positioned. Accordingly, both lateral rectus muscles were recessed 6.5 mm and supraplaced. A V-pattern exotropia persisted postoperatively with clinical overaction of the inferior obliques. To our knowledge this is the first known case of extraocular muscle abnormalities associated with schizencephaly.