Friedrich's ataxia (FA) is an autosomal recessive disorder. It is the most common cause of inherited ataxia. It affects approximately 1-2 persons per 100,000 population.1 It occurs due to a mutation that results in the homozygous expansion of Guanosine Adenine Adenine trinucleotide (GAAT) repeat units in intron 1 of FRATAXIN gene in chromosome 9.2,3 Its pathophysiology can be explained by “Dying back phenomenon”; progressive damage to axons with ultimate neuronal death, mostly in spinal cord but can also involve cranial nerves VII, X and XII.4 Apart from neurological manifestations cardiomyopathy5,6 and endocrine pathologies also occur which contribute to a worsened prognosis of the disease i.e. within 15-20 years of onset, patient becomes wheel chair bound and has a shortened life expectancy. Cardiac pathologies are the most common cause of death in Friedreich's ataxia.2