Fourth ventricular low-grade tumoral or dysplastic neuronal lesions have been reported as an epileptic focus for recently described cerebellar epilepsy in the form of repetitive and stereotyped attacks of hemifascial spasm, eye blinking, fascial movements, head deviation and dysautonomic manifestations. The case of a 3-month old infant having fourth ventricular mass with similar symptoms such as paroxysmal facial movements, eye blinking, eyelid contractions and abnormal head posture is reported in this article. After a few days of her admission, her attacks displayed a new form with altered consciousness and left limb jerks which were unresponsive to medical therapy. Following the surgical excision of the lesion 10 months ago, attacks disappeared and she is still seizure free. Histopathological diagnosis was ganglioneurocytoma. The seizures (which may be intractable in cerebellar epilepsy) are thought to have arisen from subcortical structures such as cerebellum, brain stem nuclei or the lesion itself. In the case of intractable episodes, surgical excision may prevent further seizures and help patients have a normal cognitive and motor development. (c) 2007 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.