Mitochondrial diseases increase mortality and morbidity caused by mutations in mitochondrial DNA (mtDNA) or functional gene disorders of mitochondria. Hereditary mutations in mtDNA are an important cause of genetic diseases for which there is no effective treatment and are transmitted from generation to generation. Mutations in DNA have an important role in infertility or other reproductive anomalies that occur later in life by affecting oocyte functions as well as negatively affecting vital organs. With the development of technology, one of the new treatment approaches developed for mitochondrial diseases is mitochondrial gene replacement therapy (MRT). MRT allows women who are carriers of mtDNA mutations to have a genetically healthy child. In this review, MRT, its effects, nursing dimensions, and ethical relationship are discussed.