Although being rare, Friedreich's ataxia is the most common inherited ataxia. Friedreich's ataxia causes muscle weakness, dysarthria, scoliosis and cardiac symptoms which can cause even death. These are challenging to the anaesthetist and even gets worse with the progression of the disease. Patients are sensitive to non-depolarising muscle relaxants and even regional anesthesia can be difficult due to scoliosis with also medicolegal risk. This is a case report of a 36-year-old wheel-chaired man with Friedreich’s ataxia, who underwent a right interlocking nail tibia insertion for tibia fracture. General anaesthesia with laryngeal mask airway without muscle relaxant was used and SIMV mode was the ventilation mode of choice. The patient recovery was smooth with no pain and he was transferred to the ward after 1 h in the recovery room.