Atlantoaxial rotatory subluxation (AARS) has been described for more than 100 years since it was first described by Bell in 1830. Due to the limited understanding of the disease, there are many naming controversies, such as Grise’s syndrome, atlantoaxial subluxation, spontaneous subluxation subluxation, rotational subluxation, rotational fixation, and so on. Currently, most scholars prefer to accept the concept of rotational subluxation of the atlantoaxial joint, which reflects the actual pathological changes and can be distinguished from the management of subluxation cases in clinical practice. Rotational subluxation of the atlantoaxial joint refers to the restriction or fixation of the normal movement between the atlantoaxial and the pivotal spine, often secondary to cervicopharyngeal infection, minor or severe trauma, and is a common disease in children with oblique neck. However, there has been no unified view on the diagnosis and treatment of this disease in the literature over the years, so it is necessary to review the current status of the treatment of atlantoaxial rotatory subluxation as follows. Diagnosis: Since the atlantoaxial joint rotational function accounts for more than 50% of the entire neck, the loss of atlantoaxial joint rotational function will significantly reduce the quality of life of the patient, and the atlantoaxial subluxation can be progressively aggravated, resulting in high spinal cord compression and even life-threatening. Clinical features: Atlantoaxial rotatory subluxation refers to the restriction or fixation of normal movement between the atlantoaxial and pivotal vertebrae that occurs under the action of cervicopharyngeal infection, minor or severe trauma, strain and other factors, etc. Clinically, atlantoaxial rotatory subluxation should be considered in children with acquired oblique neck who have recent history of upper sensation, cervical inflammation, cervical trauma and cervicopharyngeal surgery; its main clinical manifestations are head deviation to one side and jaw turning to the opposite side. The main clinical manifestations are head deviation to one side, jaw turning to the opposite side, refusal to actively turn the head in the acute stage, and significant pain when turning passively, resulting in limited neck movement, which is called “robin’s feeding posture”, i.e. Cock-Robin’s sign, and in a few cases, symptoms of basilar artery ischemia (dizziness, headache, nausea, vomiting, etc.) and symptoms of cervical medullary compression (weakness of limbs, gait unstable gait, etc.).