Amyotrophic lateral sclerosis is a neurodegenerative disease caused by the degeneration of upper and lower motor neurons. The disorder causes muscle weakness and atrophy throughout the body. There is an unexpected response to depolarizing or nondepolarizing muscle relaxants, and risk of perioperative respiratory failure is high. Careful monitoring and minimal doses of anesthetics are needed. We report a case of general anesthesia in a patient with amyotrophic lateral sclerosis. We used proper doses of muscle relaxant and inhalation anesthetics with neuromuscular monitoring and bispectral index. The patient early recovered from anesthesia without any respiratory complications.