Amyotrophic lateral sclerosis (ALS) is one of the major neurodegenerative diseases that involves degeneration at all levels of the motor system- from the cortex to the anterior horn of the spinal cord. Patients with ALS often have difficulty of ambulation for dental treatment though they have poor oral hygiene state. General anesthesia may cause respiratory problem due to its high sensitivity to muscle relaxant and weakened upper airway. In this case report, 38-year-old female patient with ALS required many dental treatments. Conscious sedation with intravenous target controlled infusion method was successfully employed and patient was discharged without any complications.