The treatment of upper cervical spine TB depends on the extent of the disease, neurological status and patient comorbidities. In patients with no deficit, a transoral aspiration biopsy is recommended prior to nonoperative with cervical orthosis. It is essential to ensure compliance with anti-TB medication and improve the nutritional status of the patient. Flexion and extension views are mandatory to detect and treat atlantoaxial instability. Patients with upper cervical spine TB in whom myelopathy is present due to atlantoaxial dislocation are ideally managed with transoral/retropharyngeal decompression biopsy followed by reduction and posterior stabilization, which may facilitate early neurological recovery and provide immediate spinal stability.