Head jerking is one of the most common symptoms of motor tics, and because of this, patients are at an increased risk of cervical spine disorders. However, there have been no reports of atlantoaxial subluxation in the English literature. To the best of our knowledge, this is the first case of atlantoaxial subluxation associated with chronic motor tics. A 41-year-old man with a history of chronic motor tics since childhood was diagnosed with high cervical myelopathy due to atlantoaxial subluxation. The patient underwent posterior fusion surgery using atlantoaxial instrumentation and an autologous bone graft. Although screw breakage occurred as an early postoperative instrumentation failure, the clinical outcome was excellent after surgery without recurrence of subluxation. Other techniques such as atlantoaxial transarticular fixation and occipitocervical fusion followed by long-term external immobilization might be treatment options at the initial surgery, or in case of postoperative recurrent atlantoaxial subluxation.