Background: Friedreich's ataxia is the most common hereditary ataxia and its clinical spectrum includes cardiac disease, mainly hypertrophic cardiomyopathy.
Methods: We present two cases with molecular diagnosis of Friedreich's ataxia and cardiac disease shown on electrocardiogram and echocardiogram.
Results: Neurological symptoms which lead to the diagnosis are described together with cardiac comorbidities.
Conclusions: The cases here described highlight the importance of early screening and identification of systemic complications, specifically cardiac disease, in patients with this neurological disease.