Background: We summarize the efficacy and safety data for omaveloxolone in patients with Friedreich ataxia from the MOXIe clinical trial (NCT02255435, EudraCT2015-002762-23) and post hoc analyses. Methods: In MOXIe Part 2, patients aged 16-40 were randomized 1:1 to receive omaveloxolone 150 mg or placebo. The primary outcome was change in modified Friedreich Ataxia Rating Scale (mFARS) from baseline to Week 48—patients could roll over into an open-label extension (OLE). A post hoc propensity-matched analysis compared treated and untreated patients over 3 years. Results: Treatment with omaveloxolone significantly improved mFARS relative to placebo at Week 48, with a difference of -2.41 points for the full analysis set (n=82 [excluding severe pes cavus]; p=0.01) and -1.93 points for the all-randomized population (n=103 [including severe pes cavus]; p=0.03). Transient and reversible changes in aminotransferase levels were observed with omaveloxolone without other signs of liver injury. Headache, nausea, and fatigue were among the more common adverse drug reactions in omaveloxolone-treated patients. In a post hoc propensity-matched analysis, omaveloxolone-treated patients in the OLE progressed by 3 points at Year 3 versus 6.6 points in an untreated matched cohort. Conclusions: Patients who received omaveloxolone showed a significantly stabilized neurological function and slowing of FA progression, as measured by mFARS.