Mitohondrial optic neuropathies are a heterogenous group of disorders that present with visual disturbances resultant from mitochondrial dysfunction within the anatomy of the Retinal Ganglion Cells (RGC), optic nerve, optic chiasm, and optic tract. These disturbances are multifactorial, their aetiology consisting of metabolic and/or structural damage as a consequence of genetic mutations, environmental stressors, or both. The three most common neuro-ophthalmic abnormalities seen in mitochondrial disorders are bilateral optic neuropathy, ophthalmoplegia with ptosis, and pigmentary retinopathy. Signs and Symptoms The generalized, common presentation for this broad and inclusive group of diseases is painless, bilateral loss of visual acuity and pallor of the optic disc accompanied with varying degrees of dyschromatopsia and central/cecocentral scatomas. On examination the pupillary responses may be sluggish to light. One would not expect to find an afferent pupillary defect because optic neuropathies are often bilateral and symmetric. The optic disc may appear mildly hyperemic with small splinter hemorrhages on or around the disc, or may appear nearly normal. Optic atrophy typically develops later and may appear mild. In later stages the optic atrophy can become severe, which indicates less opportunity for recovery. The duration of onset can vary between immediate and insidious, owing to the specific etiology. Two key features may be helpful in distinguishing acquired from inherited optic neuropathies: absence of a family history and simultaneous involvement of both eyes; the former more commonly characterized by these two features. Causes Because this grouping of diseases is of heterogenic origin, the causes can be singular or additive consequences of genetic, toxic, or nutritional stress. Mitochondria are maternally inherited, so a genetic defect in mitochondrial DNA (mtDNA) is passed on from mother to child. Mitochondria, however, depend on other proteins that are encoded by nuclear genes, constructed in the cytoplasm and then transported into the mitochondria. So it follows that, while an mtDNA point mutation are inherited through the mother, defects in nuclear DNA, even those affecting the mitochondria, will be transmitted in Mendelian fashion. Optic neuropathies that are acquired can be the result of several processes. These include prolonged use of certain antibiotics or anti-tuberculosis medications, exposure to certain toxic chemicals, and situations that contribute to poor consumption or decreased absorption of nutrient-dense foods. A possible synergism between genetic and acquired mitochondrial optic neuropathies has been suggested and there are only a few case reports to support this phenomenon, requiring further research and demonstration of evidence