The management of medically intractable epilepsy is frequently assisted by the identification of structural abnormalities made possible by modern imaging techniques. The association between meningoencephaloceles and epileptic seizures is well reported in the literature. We report a patient with refractory right frontal lobe epilepsy caused by a right nasal meningoencephalocele who was rendered seizure free by endoscopic nasal excision and skull base repair, obviating the need for resective epilepsy surgery. Epilepsy patterns associated with encephalocele and their management are reviewed.