The review will focus on mitochondrial diseases where AAV-based gene therapies have been pre-clinically tested in mice: Barth syndrome, Friedreich ataxia, NDUFS4, NDUFS3, and SURF1-related Leigh syndrome, ethylmalonic encephalomyopathy, three mitochondrial DNA depletion disorders (mitochondrial neurogastrointestinal encephalomyopathy (MNGIE), MPV17 deficiency, and TK2 deficiency), Leber hereditary optic neuropathy and SLC25A46-related neuropathy. Here, NDUFS4 is linked to Leigh syndrome.