A 4-month-old female infant presented with paroxysmal clonic or tonic hemifacial contractions. Afterward, she sometimes had clonic hemibody contractions. Nystagmoid eyeball movements were also occasionally seen. These evolved to stereotypic clinical patterns and frequencies, despite administration of antiepileptic drugs. Magnetic resonance imaging revealed a mass in the right cerebellar hemisphere, involving the right middle peduncles and effacing the fourth ventricles. Video-scalp electroencephalogram monitoring revealed a left fronto-temporal irregular spike slow wave that at times propagated to the contralateral hemisphere. The lesion was removed and pathologic diagnosis was ganglioneurocytoma. Postoperatively, the infant did not present any new seizures but she died eight days after surgery due to severe duodenal hemorrhage. We suggest that cerebellar ganglioneurocytoma may cause epileptic hemifacial seizures of subcortical origin as was previously described.