Atlanto-occipital deformity is a common congenital malformation disease, which refers to the developmental and structural-functional abnormalities of the base of the occipital bone, atlas, cardinal vertebrae and their surrounding soft tissues due to various factors. It mainly includes deformities such as subungual herniation of the cerebellar tonsils, flattened skull base, and atlanto-axial dislocation. The age of onset varies, and the clinical symptoms are not specific, but can manifest as numbness and weakness of the limbs, dizziness, muscle atrophy, shortness of breath, difficulty swallowing, choking on water, neck, shoulder, back and leg pain, etc. If clinicians do not understand the disease, it is easy to miss the diagnosis. Atlanto-occipital joint anatomy diagram Because patients with atlanto-occipital deformity are often combined with extensive compression of the spinal cord, they have more potential cervical marrow injury factors than normal people, so without active therapeutic intervention, patients have a high rate of paralysis and mortality. In the past, decompression of the foramen magnum alone was the standard procedure for atlanto-occipital deformity, but this procedure increases the instability of the craniocervical junction while relieving the patient’s original deformity, so the patient’s clinical symptoms may not be relieved or may even worsen. In addition to the compression of the spinal cord, the atlanto-occipital deformity still has the factor of craniocervical instability. If the surgery only releases the compression, the patient’s symptoms may be relieved for a short period of time, but the potential instability may make the patient’s vertebral body subluxation or re-injury. Occipital foramen magnum decompression combined with occipitocervical fusion not only relieves the compression to which the spinal cord is subjected, but also restores normal neck stability by internal fixation with occipitocervical fusion. The occipitocervical fixation plate has good resistance to rotation and horizontal displacement. The pedicle screws provide strong internal fixation without external fixation and require only a cervical brace for a period of time after surgery to prevent accidents. Patients are able to get out of bed a few days after surgery without complications due to prolonged absolute bed rest, whereas patients undergoing simple decompression require longer bed rest and prolonged external fixation of the surgical site after surgery. In some patients with irreversible anterior compression, such as compression of the medulla oblongata or the ventral aspect of the spinal cord by the dentate process, single-opening decompression via the posterior cervical approach is ineffective and may even aggravate the condition, and the dentate process should be removed or repositioned via the oral route, followed by posterior cranial fossa decompression and fusion. The following is a case of a female patient, 25 years old, who was sent to the emergency department of a tertiary care hospital in an emergency after a fall with quadriplegia and respiratory distress, with clear consciousness, tangential answers, labored and rapid breathing, quadriplegia, muscle strength grade 0, and sensation in the extremities. CT of head and neck suggested atlantoaxial joint dislocation, MRI of cervical spine suggested atlantoaxial joint dislocation and obvious pressure on cervical medulla. The orthopedic surgeons and neurosurgeons of the local hospital thought that the surgery was too risky and did not dare to do it. After reading the film, I analyzed that the patient was still young and had been injured for a short period of time, so it was difficult to live without surgery, and surgery was necessary to save his life. The patient should be able to walk on the ground. I told the family that there was a risk involved in the surgery, but they had to take that risk. After the operation, I gave her transcranial magnetic stimulation therapy as soon as possible. After one month, the patient’s muscle strength recovered to level 3, and after two months, she could walk on the floor with assistance, and after three months, she was completely self-sufficient. Here, I would like to tell you that patients with cervical medullary compression and paraplegia should not panic, and if they need surgery, they should operate even if they take risks.