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Background: MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, stroke-like episodes) is a rare, multisystem disorder which belongs to a group of mitochondrial metabolic diseases. As other diseases in this group, it is inherited in the maternal line.

Case Report: In this report, we discussed a case of a 10-year-old girl with clinical and radiological picture of MELAS syndrome.

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