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Friedreichs ataxia is a rare (1:50 000) autosomal recessively inherited neurodegenerative disorder. Findings such as weakness in skeletal muscles, progressive difficulty in walking and extremity ataxia are prominent. Problems such as cardiac, endocrine, pulmonary and neuromuscular dysfunction may affect the anesthesia method of patients with Friedreichs ataxia. In this case, we wanted to present our anesthetic management to a patient with Friedreichs ataxia who underwent posterior spinal fusion operation due to kyphoscoliosis. [Med-Science 2013; 2(4.000): 928-34]