Study Design. Case report and review of relevant literature. Objective. To report a rare complication of esophageal perforation in cervical fracture patient with progressive ankylosing spondylitis (AS) and review of relevant literature. Summary of Background Data. Esophageal perforation in cervical fracture is extremely rare. This complication has never been reported in AS patients. Key points in the early diagnosis and management of this severe complication along with the cervical fracture were presented in detail in this case report. Methods. A 61-year-old male with AS, who sustained C6 fracture and dislocation after a vehicle accident. Initial treatment strategy was combined anterior-posterior fixation and fusion. Although esophageal perforation in the dorsal wall of esophagus was detected in anterior surgery. After thorough debridement, the esophageal lesion was closed by resorbable interrupted sutures. Surgical incision was washed by saline and hydrogen peroxide without primary closure. Cervical fracture was treated by long level posterior fixation alone. After surgery, the anterior surgical incision was irrigated with normal saline twice a day and closed 3 weeks later. Results. Esophageal perforation was healed completely and neurologic results were improved significantly after surgery. Union of the fracture and segmental stability of cervical spine was confirmed radiologically at 1-year follow up. Conclusion. Esophageal perforation in cervical fracture patient with progressed AS is an extremely rare and potentially life-threatening complication. Surgical debridement and drainage with intensive care are essential for good outcome.