The objective of this study was to examine the expressions of mitochondria, biogenesis and mtDNA genes on sports performance in many aspects. The study included the summarization of the studies registered in Pubmed Central, Pubmed and Google Scholar internet database. Sporting performance is a multifactorial phenomenon that is affected by many factors. Genetics, which are candidates to be one of these factors, can have a significant power on sports performance. So far, 239 genetic markers have been identified for the relationship between sports and genetics. Of these, 214 are automosal, 18 mitochondrial, and 7 are localized on the X chromosome. mtDNA has both fewer nucleotides and a smaller amount of genes compared to DNA in the nucleus. Mitochondria, which are the power plant of the cell, can activate some genes in mtDNA in the synthesis of ATP by oxidative phosphorylation. In the current study, it was determined that the activities of the genes in mtDNA on sports performance are actually the contributions of aerobic energy pathways to performance. Mitochondrial biogenesis may improve endurance capacity in sports by providing the formation of low and moderately reactive oxygen species. On the contrary, excessive production of reactive oxygen species may cause some functional disorders in mitochondria in parallel with the increase in oxidative stress. As a result, genes in mtDNA can have important effects on endurance performance in sports. Conducting clinical studies based on robust methodologies in this field will make valuable contributions to sports sciences.