The scarcity literature addressing the cardiovascular effects omaveloxolone in FRDA is concerning, especially considering that cardiomyopathy is the primary cause of mortality in affected individuals. Furthermore, the reported potential of omaveloxolone to induce mil elevations in B-natriuretic peptide (BNP), a known marker of heart failure, adds to this concern. Thus, those individuals with FRDA who have cardiomyopathy are advised to monitor BNP levels while using omaveloxolone. In this viewpoint, we examine the current literature on the efficacy and safety of omaveloxolone in treating FRDA, with a focus on its potential impact on cardiovascular health.