Tuberculosis of the craniovertebral junction is an uncommon entity and its optimal management remains controversial. In this study, we present the evolution of management protocol of this disease in our institute in the past 3 decades. A total of 51 patients with craniovertebral junction tuberculosis presenting as atlantoaxial dislocation from 1978 through 2004 were reviewed. The disease was rated from Stage I to Stage III, depending on the radiological findings. All patients received antitubercular treatment for 18 months. In the initial period of this study (1978-1986), all patients (n = 10) underwent surgery, usually a posterior fusion. In the second period (1987-1998), patients with less severe disease (Stages I and II, n = 14) were managed with external rigid immobilization, whereas patients with severe disease (Stage III, n = 11) underwent either a transoral decompression with or without posterior fusion or posterior fusion alone. More recently (1999-2004), all patients (n = 16) in all stages (Stages I-III) have been managed without surgery by a rigid external immobilization. Except for two patients who died (one because of miliary tuberculosis, the other because of acute hydrocephalus), clinical recovery occurred in all. Follow-up imaging demonstrated radiological healing as well, with regrowth of the destroyed bone. The mainstay of management of tuberculosis of the craniovertebral junction is prolonged antitubercular treatment with a rigid external immobilization. Surgery is not necessary, even in patients with advanced stages of disease. Complete clinical and radiological healing occurs in all patients with conservative treatment.