Tuberculosis of the craniovertebral junction is extremely rare. However, recent evidence suggests that the incidence of this condition may be increasing in the United Kingdom. The diagnosis is often difficult despite advances in imaging using MRI. CT guided biopsy of lesions often yields inconclusive results. The transoral approach to the anterior craniovertebral junction provides excellent access to this region with a low operative morbidity and mortality, enabling biopsy of lesions and decompression of the neuraxis. Management of secondary atlantoaxial instability, regarding both timing and method of stabilization, is controversial. We report two cases of tuberculomas of the craniovertebral junction, that illustrate the role of transoral surgery in both diagnosis and treatment of this condition. Previous management strategies are reviewed and future recommendations are presented.