Mitochondrial DNA mutation is associated with a variety of diseases. Although only constituting 10% of the entire mitochondrial genome, the transfer RNA (tRNA) genes have become a hotspot in the study concerning the relationship of the mitochondrial gene mutation with diseases. Previous studies on the genetics of hypertension mainly concentrated in the nuclear genes, but recent studies found that mitochondrial DNA mutations may be involved in the occurrence and development of essential hypertension (EH). What’s more, much evidence indicated that close association was found between mitochondrial tRNA with the EH occurrence and development. In this article, we summarized the relationship between the mitochondrial tRNA mutation and pathogenesis of hypertension, and reviewed the underlying mechanism.