Objective: The purpose of this study is to present an ankylosing spondylitis (AS) patient with fracture-dislocation of C7/T1, who got reduction and fixation successfully by surgical treatment. Methods: A 36-year-old Chinese male with 22-years history of AS was admitted to our Emergency Department (ED) complaining about sensory and motor disturbance for 12 hours due to the fall at home. AS and fracture-dislocation of C7/T1 were diagnosed by personal history, neurological examination and X-ray, CT and MRI. Results: Posterior cervical instrumentation and fusion from C5 to T3 (lateral mass screws of C5, C6 and pedicle screws of C7, T2, T3). Meanwhile, satellite rod was placed in C7-T2 to disperse stress. The lamina and ossified ligamentum flavum of both C7 and T1 were removed for decompression; no extra-dural hematoma was observed. Given the patient's longstanding history of AS, the fracture-dislocation site was highly unstable, combined anterior and posterior fixation was performed. Then, the patient underwent open reduction, internal fixation of C7-T1 fracture and anterior cervical plating with iliac crest autogenous bone grafting. The patient did well postoperatively, his neurological state recovered to Frankel D, with muscle strength of 3 of 5 in the upper extremities and 3+ of 5 in the lower extremities. Although neurological improvement was minor, his motivation increased, and pain from his neck disappeared. Conclusion: Surgical treatment of AS patients with subaxial unstable fracture-dislocation can be performed successfully.