We describe the anaesthetic management of a 20 years old female with a Friedreich's ataxia for curettage of the uterus. After the premedication with thalamonal, diazepam and atropine the anaesthesia was induced with thiopental. Isoflurane and nitrous oxide were used for maintenance. We review the literature about this disease and its implications in anaesthesia. Anesthetic hazards to the patient with Friedreich's ataxia include potential risk of cardiac dysrhythmias and heart failure and also marked sensitivity to muscle relaxants. Respiratory complications and diabetes mellitus are other main problems in postoperative period. We conclude that this patients should be careful monitored specially cardiovascular function and neuromuscular transmission during and after anaesthesia.