In recent years, more and more mtDNA mutations related to MELAS syndrome were detected, such as A3252G, T3271C, T3291C and G3959A.[6] m.10158T>C is located in the loop domain of subunit (MT-ND3) of mitochondria complex I. It is rarely reported about the relationship of m.10158T>C mutation with MELAS syndrome. This evidence concerns the gene MT-ND3 and MELAS syndrome.