Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a rare mitochondrial disorder. Seizures, headaches, nausea and vomiting, exercise intolerance, and ragged-red fibers may be seen. In most MELAS syndrome patients, a point mutation at nucleotide 3243 encoding transfer RNA for leucine has been found. A 28-year-old woman who lost consciousness was brought to emergency room. She had a history of migraine-like headaches and secondary amenorrhea for 7 years, hearing loss for 5 years, and complex partial seizures for 3 months. She had a subacute subdural hematoma of left temporo-occipital region and subacute infarcts in cortical and subcortical areas of the left temporal lobe. Based on her history, physical and neurologic examinations, neuroimaging, and histopathologic and mitochondrial DNA analyses, she was diagnosed as having MELAS syndrome. The most outstanding feature in this case was the presence of a subdural hematoma. To the best of our knowledge, this unusual finding has not been reported previously in the literature.