Mitochondrial myopathies are a group of rare disorders caused by mutations in mtDNA or nDNA and are inherited maternally or, less commonly, following Mendelian inheritance patterns. Since mitochondria are the main source of energy in the cell, their dysfunction typically presents in tissues with high energy demands, such as the nervous system, skeletal muscles, and heart. The number of affected organ systems and the degree of damage can vary widely and can mimic a broad range of other diseases, but patients generally present with a group of symptoms corresponding to a specific syndrome. The most common phenotypic syndromes seen in clinical practice are MELAS and LHON. The diagnostic workup of mitochondrial myopathies includes a medical history focusing on the family history of the disease, physical examination, laboratory tests, and a multisystem assessment to determine potential damage to individual organs. The definitive diagnosis is based on muscle biopsy and molecular genetic testing. The therapeutic approach to mitochondrial disorders is primarly symptomatic, involing adherence to a specific dietary regimen, physical activity, intake of vitamins and antioxidants, and avoidance of medications that impair mitochondrial metabolism. In addition to treatment, prevention plays a crucial role, which encompasses genetic counseling. This involes identifying families with a history of mitochondrial pathologies and providing guidance on reproductive options. Numerous studies are currently underway on different molecules that potentially can be used in the treament of mitochondrial disease, therefore significant progress in the management of these patients can be expected.