Atlantoaxial subluxation in children is a common disease in pediatric orthopedics, mostly seen in children under 12 years old. Children’s neck muscles are weak, the cervical vertebrae and synapses are not fully developed, the atlantoaxial capsule and ligaments are lax, and there is no intervertebral disc between the atlantoaxial vertebrae, which is less stable and easily dislocated under external forces or spontaneously dislocated due to pathological reasons. Acute atlantoaxial subluxation in children often occurs after minor trauma, or after a week of upper respiratory tract infection, pharyngeal pain, or neck infection. Early clinical manifestations are sudden onset of oblique neck, pain, and limited cervical motion. Some children also have no obvious pain, only sudden squint and limited movement. Chronic atlantoaxial subluxation (e.g., atlantoaxial subluxation caused by recurrent throat infections and years of incorrect posture) lacks specific symptoms and is characterized only by intermittent neck discomfort in the early stage, which is relieved by dorsal extension and rotation of the neck and is often ignored by parents who mistake it for a bad habit. Gradually, dizziness, headache, nausea, vomiting and other symptoms may appear; when the C2 spinal nerve is stimulated, it may manifest as abnormal sensation in the occipital region, neck pain, restricted movement, individual orbital distension and blurred vision; a few manifest as rapid twitching and uncoordinated movements of simple muscle groups in the head, face and upper limbs, often appearing involuntary and difficult to control blinking, shaking head, shrinking neck, shrugging shoulders, making faces, etc., and pediatric tic disorder. It is difficult to distinguish from pediatric tics. It is also believed that 70% of children with tics have atlantoaxial subluxation. Acute atlantoaxial subluxation in children is not difficult to diagnose, while chronic patients often do not receive a correct early diagnosis and delay treatment. Atlantoaxial subluxation in children may affect the growth and development of the child or endanger the life of the child, so early diagnosis and treatment is very important, and the earlier the disease is treated, the better the results. The traditional cervical lateral and cervical open radiographs are of great importance in the diagnosis of atlantoaxial subluxation in children and are still an indispensable basis for clinical diagnosis. The open radiographs can clearly show whether the distance between the odontoid process and the lateral block of the atlantoaxial spine is symmetrical, whether the joint gap between the lateral blocks of the atlantoaxial spine is consistent, and whether the alignment between the lateral blocks of the upper cervical spine is accurate; the lateral radiographs can clearly show the distance between the odontoid process and the anterior arch of the atlantoaxial spine. distance between the atlas and the anterior arch. However, the diversity and complexity of trauma mechanisms and the special characteristics of children’s bones make atlantoaxial dislocation complicated, and the traditional X-ray can no longer fully reflect the nature of dislocation, the degree of dislocation, and the combined bone, soft tissue, and spinal cord damage. The atlantoaxial joint can be reproduced in coronal, sagittal and transverse axial positions, which provides the most intuitive basis for determining atlantoaxial dislocation; and In children with cervical or occipital pain or oblique neck, CT scan of the atlantoaxial joint should be performed routinely if there is no abnormality in plain film and CT scan of the resting position to exclude atlantoaxial joint instability. Non-surgical treatment remains the first choice of most orthopaedic surgeons if the disease is diagnosed early before the onset of spinal symptoms. Maxillo-occipital band traction and cervical brace fixation is the preferred treatment option for acute atlantoaxial subluxation in children. Its operation is simple, safe and reliable. Cranial traction and external fixation of the cervicothoracic brace can also be considered in some cases with long duration and stubborn lesions. Mild atlantoaxial subluxation does not require surgery even if it is combined with mild compression of the dural sac. Early surgery is required only if the bone and ligament complex is damaged and the atlantoaxial joint becomes persistently unstable or is accompanied by significant neurological symptoms. Occipitomandibular traction is appropriate in the prone position. The treatment is done in a supine position with a cushion on the back of the neck, the thickness of which is about 2 to 3 cm, and the sling is placed around the lower jaw and the occipital ridge respectively, so that the cervical spine is in a mildly hyperextended position or a neutral position, and traction is applied in the direction of the head. The traction weight is 1 to 3 kg, generally not exceeding 10% of the child’s body weight. In the past, continuous traction was mostly used, but continuous traction is more painful and difficult for the child to tolerate, and compliance is poor. At present, intermittent traction is often used, with 30-40 minutes of traction each time, twice a day. When the child is out of bed during non-traction time, a neck brace is worn for immobilization. During the treatment period, the child should rest in bed as much as possible and avoid strenuous exercise. Traction is usually applied for 1-2 weeks, and a neck brace is used to immobilize the patient for 1-2 weeks after repositioning. Antibiotics or antiviral drugs should be used for those with infection. Acute atlantoaxial subluxation in children is not difficult to treat, and most patients can be reset after 1-2 weeks of traction. It is not uncommon for the treatment to last more than two weeks. Chronic atlantoaxial subluxation in children is more common in children over 6 years of age and less common in those under 6 years of age. Chronic atlantoaxial subluxation in children has a long course and requires a relatively long treatment and rehabilitation time, often 2-3 weeks or even longer. Most of them need to be repositioned with orthopedic manipulation in order to achieve good results. Early traction treatment and one-time complete recovery are extremely important, and the treatment should be consolidated and reviewed regularly after discharge from the hospital. Cervical brace fixation, especially when there is inflammation of the upper respiratory tract, can well prevent recurrence and avoid surgical treatment such as cervical fusion. The non-surgical treatment of this disease is reliable, and surgical treatment is rarely required. The indications for surgery are (1) the presence of nerve injury; (2) significant anterior displacement of cervical 1; (3) deformity for more than 3 months and failure of conservative treatment; and (4) recurrent deformity after at least 3 months of conservative treatment and fixation. Atlantoaxial joint in children should not be neglected, especially spontaneous atlantoaxial subluxation, which mostly occurs after respiratory infections, but also in some children due to excessive use of electronic products and improper posture. The diagnosis of atlantoaxial subluxation still needs to be refined, as imaging is not the gold standard and needs to be combined with symptoms and signs, otherwise many cases will be missed and misdiagnosed. As an interdisciplinary and borderline condition, atlantoaxial subluxation requires the attention of internal medicine and pediatrics and the collaboration of spine specialists to avoid misdiagnosis and delayed treatment.