Friedreich ataxia (FRDA) is the most common inherited ataxia, with an incidence ranging from 1:20 000 to 1:250,000, or less depending on countries.1,2 Genetic transmission is autosomal recessive and is caused by mutations in the frataxin gene (in most cases a GAA repeat expansion in Intron 1) leading to reduced expression of frataxin.3,4 Symptoms include limb and gait ataxia, dysarthria and cardiomyopathy.5 Onset is typically in the second decade of life,6 and there is currently no effective disease-modifying treatment. The gene discussed is FXN; the disease is Ataxia.