Omaveloxolone was recently approved by the U.S. Food and Drug Administration (FDA) as the designated treatment for Friedreich ataxia (FRDA),1 an autosomal recessive disorder caused by mutations in the frataxin gene. FRDA is characterized by ataxia and cardiomyopathy.2-4 The pathogenesis of FRDA can be attributed to frataxin deficiency, which leads to increased oxidative stress, impaired nuclear factor erythroid 2-related factor 2 (Nrf2) signaling, dysregulated metal homeostasis, and chronic inflammation.5, 6 Omaveloxolone is an activator of Nrf2, which aims to counteract these pathologies by enhancing the expression of antioxidant enzymes and restoring redox balance, which is dysregulated in FRDA.7, 8 Although both preclinical and clinical studies have shown promising outcomes regarding neurological function, the impact of omaveloxolone on cardiovascular outcomes in FRDA remains unclear. The scarcity of literature addressing the cardiovascular effects of 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 mild elevations in B-natriuretic peptide (BNP),9, 10 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.9 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.