Tuberculosis (TB) rarely involves the craniovertebral junction (CVJ). Atlantoaxial dislocation (AAD) is one of the most commonly encountered lesions in craniocervical TB. The incidence of TB and its craniovertebral manifestation is increasing even in developed countries because of intercontinental migration and increased prevalence rates of immunosuppression conditions. While the treatment of craniovertebral TB is well standardized and relies on conservative measures, the treatment of TB with AAD is disputable. In this paper we present a review of the literature and elucidate our approach to craniovertebral TB with AAD through a case illustration.