Schizencephaly is an extremely rare congenital structural disorder presented in 1.54/100,000 births. In this article, we present one of the few cases of schizencephaly reported in Colombia, in which the linguistic barrier and the lack of resources made the differential diagnosis a challenge. The patient presented to the emergency department with focal impaired-awareness seizures associated with an inability to speak since birth. On physical examination, it was evident the loss of cephalic support, mental retardation, and left- sided hemiparesis for which a non-contrast brain computed tomography was performed showing findings that are in relation to open- lip schizencephaly (right frontoparietal brain cleft lined by gray matter which is connecting the subarachnoid space to the ventricular system, polymicrogyria and absence of septum pellucidum). Neuro-rehabilitation and antiseizure therapy were established with good outcomes. It is important to understand that nowadays does not exist any curative procedure, the mainstay of treatment is based on neuro- rehabilitation which should be initiated as soon as possible because it improves the cognitive and motor status of patients.