A patient with Friedreich's ataxia was hospitalized due to dilated cardiomyopathy and heart failure. We received informed consent from the patient and his family for implantation of an implantable ventricular assist device (VAD) as permanent support. At the one-year follow-up examination, the neuromuscular symptoms had progressed no further, and he had no VAD systemic failure and complications. This is the first case report of VAD implantation in a Friedreich's ataxia patient.