Amyotrophic lateral sclerosis (ALS) is a progressive, neurodegenerative disorder involving primarily motor neurons in the cerebral cortex, brain stem and spinal cord, which leads to respiratory failure and death. The impairment of respiratory function, together with weakened upper airway muscles, may affect anesthetic management. The response to muscle relaxants, either depolarizing or nondepolarizing, is also altered in patients with ALS. Patients with ALS require special care throughout the perioperative period. We experienced the anesthetic management of a 63-year-old female patient with ALS under general anesthesia without the use of muscle relaxants for closed reduction with percutaneous screw fixation to treat a right femur neck fracture.