pinocerebellar ataxia (SCA) is an autosomal-dominant inherited neurodegenerative disorder characterized by various slowly progressive symptoms of ataxia, such as ataxic gait and dysarthria. 1 Although some patients with SCA have undergone surgery under general anesthesia, orthognathic surgery in patients with SCA is unreported. Here, we report a patient with SCA type 3 (SCA3) who underwent orthognathic surgery to correct a mandibular protrusion with facial asymmetry.