The MELAS (Mitochondrial Encephalomyopathy with Lactic Acidosis, and Stroke-like episodes) syndrome is one of the inherited mitochondrial disorder. We have experienced a 16-year-old girl with headaches and left hemianopsia. Diagnosis of MELAS syndrome with multiple brain parenchymal lesions was confirmed by gene study. The stroke-like lesion of MELAS syndrome showed significant improvement in radiological follow up study. Therefore, MRI findingsin MELAS could be interpreted as metabolic cellular dysfunction rather than ischemic vasculopathy
