Friedreich’s ataxia (FRDA) is the most common autosomal recessive form of ataxia that affects approximately 1 in 50,000 Caucasians. FRDA is caused by autosomal recessive GAA trinucleotide expansion in the first intron of the FXN gene on the proximal long arm of chromosome 9, which interferes with frataxin transcription. Moreover 1–3% of the patients are heterozygosus for the GAA expansions on one allele and a point mutation or deletion on the other allele of FXN. Typical age at onset occurs around or even before puberty, although showing a very large variability even between siblings. Progressive gait ataxia, dysmetria, dysdiadocokinesia, muscular weakness, sensory loss, areflexia are typical clinical features of the disease. In addition to neurodegenerative symptoms, there is a multiple systemic involvement that includes cardiomyopathy, scoliosis, diabetes mellitus, foot deformities, abnormalities of eye movements. FRDA patients are wheelchair bound by a disease duration of 15.5 years (range 3–44) with a shortened life expectancy .