Surg Neurol
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Tuberculosis of the craniovertebral junction (CVJ) is extremely rare. However, recent evidence suggests that the incidence of this condition may be increasing. The diagnosis is often difficult despite advances in imaging using magnetic resonance imaging. The transoral approach to the anterior CVJ provides excellent access to this region, has low mortality and morbidity, and enables biopsy of lesions and decompression of the neuraxis. Management of associated atlantoaxial instability, with regard to timing and method of stabilization, is controversial. ⋯ Although CVJ tuberculosis is a rare disease, the outcome of treatment is good. Antituberculous drug therapy remains the mainstay of treatment after confirming the diagnosis. The surgical management options include transoral decompression with or without posterior fusion, depending upon the presence and persistence of atlantoaxial instability.