Amyotrophic lateral sclerosis (ALS) is a degenerative disease of motor ganglia in the anterior horn of spinal cord and spinal pyramidal tracts. The impairments of respiratory function and weakened upper airway muscle affect anesthetic management. General anesthesia may cause respiratory problem. Regional anesthesia such as spinal and epidural anesthesia is also relative contraindication for patients with a motor neuron disease, including ALS, for the fear of exacerbating the disease. Therefore patients with ALS are needed careful management during perioperative period. We report a case of epidural anesthesia for transurethral ureteroscopic lithotripsy in a patient with ALS. The patient recovered from anesthesia without exacerbating the disease.