Craniovertebral junction (CVJ) tuberculosis is a rare disease, and potentially fatal due to the risk of atlantoaxial dislocation. The disease usually accompanies a retropharyngeal cold abscess, which can cause subsequential otolaryngologic symptoms such asdysphagia, odynophagia, or airway obstruction. Such a patient must be handled with great care to avoid a disaster. The disease is diagnosed with microbiological or other laboratory tests on the pus collected through puncture and aspiration, which will need otolaryngologic skills. For treatment, otolaryngologists play an important role by doing incision and drainage of the retropharyngeal abscess, or by attending the transoral vertebra surgery as partners with spine surgeons who will manage the CVJ lesions and ensure the craniocervical stability.