Pott's disease of the craniovertebral junction is extremely rare. The authors studied the immediate and long-term outcome after transoral decompression, occipitocervical stabilization with fusion and antitubercular therapy (ATT) in patients who had neurological deficits due to craniovertebral junction (CVJ) tuberculosis. This is a retrospective study of the management and outcome in 10 consecutive patients in whom features of spinal cord (with or without) compression were observed, and CVJ tuberculosis was diagnosed in two different neurological departments between 1990 and 2002. They ranged in age from 15 to 72 years. The onset of symptoms was either acute or subacute. Patients presented with neck and occipital pain (90%), progressive tetraparesia (40%), sensory symptoms (40%), neck stiffness (30%) and urinary dysfunction (20%). The disease caused spinal cord compression in six patients, atlantoaxial dislocation in five patients, basilar impression in one and upper vertebral column destruction without dislocation in three. Two patients presented with multiple spinal tuberculosis. Antitubercular therapy was used in all cases for 15 months. Surgical treatment performed in all patients. Four patients underwent transoral drainage of retropharyngeal abscess with granulation tissue and one odontoidectomy. Six patients then underwent occipitocervical (occiput -C3 or -C4) fusion with insertion of titanium plate and screw. Postoperatively, ATT was prescribed for 15 months. At long-term follow-up (median 50.2 months), functional status considerably improved. Two patients died 2 months later due to myocardial infarction. Patients with CVJ with features of cervical myelopathy are ideally treated with transoral decompressive procedures followed by occipitocervical fusion, because this therapy provides immediate neurological improvement, stability and allows early mobilization. The long-term prognosis in patients with this disease is excellent, provided it is treated with appropriate surgical intervention and with adequate duration of ATT. This approach provides excellent access to this region, with a low operative morbidity and no mortality.