Essential hypertension (EH) is a major risk factor for cardiovascular and cerebrovascular diseases, and is seriously harmful to human health. Previous studies have noted that there is maternal inheritance of EH in some pedigrees. Consequently, mutations in mitochondrial DNA (mtDNA) have become a new target for the pathogenesis of EH. Currently studies have found many of mtDNA mutations associated with EH. These mutations have been confirmed to lead to the failure of oxidative phosphorylation function, deficit in ATP synthesis, increase in reactive oxygen species (ROS) and mitochondrial-mediated cell death. Therefore, the further study on mitochondrial dysfunction will provide new insights into the molecular mechanism of maternal inheritance of EH. These mtDNA mutations should be considered as genetic markers for future molecular diagnosis of maternally inherited EH. The present review summarized the mtDNA mutations associated with EH and the related molecular mechanism for EH.