A case of atlanto-axial subluxation in a 17 year old boy with rheumatic disease is reported. The subluxation could not be reduced by cervical traction. Posterior fixation with acrilic and wire was unable to prevent the appearance of signs of spinal cord compression. The odontoid process was removed through a transoral approach leading to neurologic recovery. Treatment of atlanto-axial subluxation is discussed, enphasis being given to the indications for anterior descompression.