<p>This review aims to investigate the potential role of estrogen in various mitochondrial diseases, such as Leber’s Hereditary Optic Neuropathy and Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes, focusing on its effects on aging, oxidative stress, mitochondrial biogenesis, and mitophagy. Mitochondrial diseases have become important in modern medical research due to their complex genetic background and diverse clinical manifestations. Studies in recent years have shown that estrogen plays an essential role in physiological regulation and may also affect the health status of cells by regulating mitochondrial function, which in turn affects the occurrence and development of diseases. However, there is still a lack of systematic review of estrogen’s specific mechanisms and roles in these diseases. This review will synthesize the relevant literature to explore the association between estrogen and mitochondrial diseases and its possible therapeutic prospects, aiming to provide a theoretical basis and reference for future research.</p> Graphical Abstract <p>An overview of how estrogen affects mitochondria. Estrogen maintains mitochondrial function and alleviates the aging process. Besides, it has potential therapeutic effects in LHON and MELAS by regulating cellular functions.</p> <p></p>