Spinocerebellar ataxia type 1 (SCA1) is one of the autosomal dominant spinocerebellar degeneration (SCD) diseases characterized by progressive cerebellar ataxia, muscle atrophy, and peripheral neuropathy. We report the management of a 43-year-old man with SCA1 who underwent general anesthesia for open reduction and internal fixation of a mandibular fracture. Although anesthesia-induced vocal cord paralysis has been reported in patients with SCD, nasotracheal intubation was performed uneventfully with video laryngoscope. After taking into consideration the increased risk of postoperative respiratory depression in patients with SCD, rocuronium dosing was titrated carefully, and fentanyl was not used during surgery. Preparation for an anticipated difficult airway and avoiding significant respiratory depression are crucial when providing general anesthesia for patients with SCA1.