Objective To investigate the safety and clinical efficacy of microendoscopically monitored anterior transoral pharyngeal approach with anterior release and posterior pedicle screw internal fixation for the treatment of occipital and cervical deformity. Methods From February 2009 to July 2013, a total of 13 cases of refractory atlantoaxial subluxation combined with high cervical spinal cord compression were treated by microendoscopically supervised anterior transoral pharyngeal release and one-stage posterior atlantoaxial pedicle screw internal fixation with bone graft fusion. There were 8 male and 5 female cases; ages ranged from 26 to 55 years, with an average age of 46 years; disease duration ranged from 3 to 5 years. There were 3 cases of old odontoid fracture and 10 cases of congenital malformation. All patients had different degrees of spinal cord compression symptoms. The preoperative JOA scores ranged from 7 to 12, with a mean of 9.1. All patients underwent transoral anterior atlantoaxial release, one-stage posterior atlantoaxial screw system repositioning, internal fixation, and bone graft fusion. The operative time, bleeding volume and postoperative complications were recorded, and the clinical efficacy was followed up with X-ray, CT, MRI and other imaging examinations to observe the repositioning, internal fixation and bone graft fusion. Results Intraoperative bleeding volume, time, and no intraoperative vascular and nerve injury. Follow-up was obtained in all patients for 12-36 months, and all patients obtained bony fusion with no internal fixation failure or loss of repositioning 6-12 months after surgery. Postoperative neurological function improved significantly with a JOA score of 11-16, with a mean score of 14.5. Conclusion The treatment of refractory atlantoaxial subluxation by microendoscopic surveillance via oropharyngeal anterior release and repositioning with posterior pedicle screw internal fixation has the advantages of clear surgical view, precise operation, and less injury on the basis of ensuring good clinical efficacy.