Tuberculosis of the craniovertebral junction is extremely rare. However, re
cent evidence suggests that the incidence of this condition may be increasi
ng in the United Kingdom. The diagnosis is often difficult despite advances
in imaging using MRI. CT guided biopsy of lesions often yields inconclusiv
e results. The transoral approach to the anterior craniovertebral junction
provides excellent access to this region with a law operative morbidity and
mortality, enabling biopsy of lesions and decompression of the neuraxis. M
anagement of secondary atlantoaxial instability, regarding both timing and
method of stabilization, is controversial. We report two cases of tuberculo
mas of the craniovertebral junction, that illustrate the role of transoral
surgery in both diagnosis and treatment of this condition. Previous managem
ent strategies are reviewed and future recommendations are presented.