Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a syndrome which is characterized as mitochondrial myopathy, encephalopathy, lactic acidosis and recurrent stroke-like episodes. Recurrent attacks of prolonged migrainous headache, different types of epileptic seizures and repeated cerebral lesions are the main clinical features of the disease. Cerebral lesions can cause different seizure types in this syndrome according to affected brain areas. Herein, we are reporting a case of MELAS who experienced recurrent neurologic deficits, confusional states and epileptic seizures with ictal epileptic nystagmus. Ictal electroencephalogram (EEG) recordings and magnetic resonance imaging (MRI) lesions also supported to the ictal focus of epileptic nystagmus. With this case, we would like to take attention to this rare ictal event.