Spinal muscular atrophies are inherited neurodegenerative disorders affecting anterior horn cells. There are various problems, especially weakness of respiratory muscle and abnormal reaction to muscle relaxants during the general anesthesia. And gingival hyperplasia can make the proper airway management difficult. Experience with anesthetic management in a patient with spinal muscular atrophy combined with gingival hyperplasia has been very rare. We report the anesthetic experience of a wheelchair-bound child, who underwent gingivectomy under general anesthesia. The child was safely managed with fibroscopic nasotracheal intubation under sevoflurane without muscle relaxants. Also, there was no deterioration of her underlying neurologic conditions.