We present a case report of a 27-year-old man with MELAS, who presented with general convulsions and left flaccid hemiparesis. Anticonvulsant drugs failed to achieve complete control of his convulsions. A good response to oral administration of succinate has been maintained for more than 30 months, with no recurrence of any stroke-like episode. Succinate therapy may have potential for treatment of uncontrolled convulsive MELAS patients.