The majority of patients with Friedreich ataxia present with gait ataxia. Congestive heart failure usually is a terminal event. We report a 9-year-old boy who developed congestive heart failure and thrombus formation in the left ventricle at age 5 years and then progressive ataxia as well as other features of Friedreich ataxia; therefore, congestive heart failure and thrombus formation may rarely be the initial findings in Friedreich ataxia.