The condition that now bears his name was first described by Nicolaus Friedreich in a series of papers between 1863 and 1877. He noted the onset at around puberty of ataxia and dysarthria; sensory loss and weakness developed later. The skeletal deformities of pes cavus and scoliosis were also reported. Over the following generations there was a tendency to lump the inherited ataxias together, and the essential features of Friedreich’s ataxia became diluted. Clinical studies in the 1970s and 1980s1 2 and subsequent genetic studies have helped clarify these features, and Friedreich’s ataxia is now known to be the commonest of the inherited ataxias, accounting for at least 50% in most large series and affecting approximately one in 50 000 individuals.3 Although at present it is an incurable and progressive disease, recent identification of the affected gene has not only provided a highly sensitive and specific diagnostic test, but has also given useful insight into the cellular pathology which may lead to the development of effective treatment.