An 18-year-woman was referred with seizure activity and global aphasia. Diagnosis of MELAS syndrome with left temporo-parieto-occipital infarction was confirmed by gene analysis. Global aphasia was improved completely. Right temporal infarction developed after 5 months. Diagnosis of pure word deafness was made in view of impaired verbal comprehension in presence of adequate reading, writing, spontaneous speaking, and well preserved environmental sound perception. MRI findings of the patient support the hypothesis of the pathway of non-verbal hearing in previous reports.