Schizencephaly is a rare congenital malformation of the brain that could present with repeated seizures. We report a case of a 17-year-old boy who presented with status epilepticus. Further radiological evaluation revealed unilateral open lip schizencephaly. Seizure was well controlled with anti-epileptic drug and there was no need for surgical intervention. This case highlights the need for prompt radiological evaluation of patients with seizures, especially in resource-limited settings where the high cost and non-availability of such facilities might be a major impediment.