Friedreich ataxia is a slowly progressive neurological disorder leading to loss of ambulation, cardiomyopathy and in so people vision loss. It is associated with mitochondrial dysfunction. This study assessed whether the mitochondrially targeted drug elamipretide might benefit those with advanced cardiomyopathy of vision loss. We randomized 16 subjects to open label treatment with either high dose or low dose elamipretide and evaluated the effect at 52 and 104 weeks. Treatment was generally safe with few serious adverse events ( none related to drug); the major adverse events were injection site reactions as described previously for elamipretide. The efficacy results were confounded by a high discontinuation rate not related to adverse events, with 50% of subjects discontinuing by 104 weeks. There was no difference in benefit on vision between subjects on low vs high dose, but comparison to a matched natural history cohort suggested some stabilization of disease. In addition, frataxin levels increase in peripheral blood and subjects on average felt vision was improved. Overall, elamipretide was safe and reasonably tolerated, but no objective evidence suggested a benefit on progression of vision loss in advanced FA patients.