Atlantoaxial position is dangerous, anatomical structure is fragile, congenital malformation, trauma, tumor, inflammation and other factors can destroy its stability and threaten the safety of the medullary life center. Ankylosing spondylitis is a chronic inflammatory rheumatism characterized by ankle arthritis and progressive spinal rigidity. The natural course of the disease often begins from the ankle and lumbar vertebrae, and then spreads to the thoracic and cervical vertebrae. Late stage of the disease may involve the upper cervical region, causing atlantoaxial dislocation. Although the incidence of the disease is not high, the onset is concealed and progresses rapidly, which can cause severe nerve compression and cause death. This article reviews the current research status of the disease.