Hip involvement in ankylosing spondylitis (AS) is a common and disabling problem. The clinical and x-ray records of 87 patients with definite AS (Rome criteria) were examined to define and characterize their hip disease. Clinical hip disease was present in 33 cases (38%), was usually bilateral (91%), and tended to begin early in the disease course; it was the cause of 50% of the Class III and IV disability in the entire study group. Typical findings included regional pain, limitation of motion, muscle atrophy, and flexion contractures. Radiologic hip abnormalities occurred in 42 cases (48%). The radiographic pattern was distinctive when compared to that in two control groups and included axial migration of the femoral head (63%), concentric joint space narrowing (50%), rufflike femoral osteophytosis (36%), and protrusio acetabuli (30%). Eight patients required bilateral hip surgery. Para-articular ossification occurred in 8 of 16 replaced hips; in 5 of 8 hips it caused clinical immobility. This potentially serious complication may limit the usefulness of hip arthroplasty in some AS patients.