MELAS syndrome(mitochondrial myopathy, encephalopathy, lactic acidosis and stroke like episodes) is a multisystem disorder characterized by short stature, vomiting, headache, cortical blindness, deafness and dementia. Point mutations of mitochondrial DNA is characteristic for this syndrome. It is inherited by maternal inheritance.

We reported two brothers who experienced MELAS syndrome with a mother whose symtom developed later.

Keywords :MELAS syndrome, Mitochondrial myopathy, Encephalopathy, Lactic acidosis, Stroke