Rotational and flexion deformity of C1-C2 due to ankylosing spondylitis is rare. We did surgical correction in one such case by lateral release, resection of the posterior arch of C1 and mobilization of the vertebral arteries, wedge osteotomy of the lateral masses of C1 and internal fixation under general anesthesia. There were no vascular and neurological complications during the surgery. After operation the atlantoaxial rotational deformity was corrected and the normal cervical lordosis was restored. At 1 year followup his visual field and feeding became normal and internal fixation was stable.