The ankylosed spine is prone to fracture after minor trauma due to its changed biomechanical properties. Although many case reports and small series have been published on patients with ankylosing spondylitis (AS) suffering spine fractures, solid data on clinical outcome are rare. In advanced diffuse idiopathic skeletal hyperostosis (DISH), ossification of spinal ligaments also leads to ankylosis. The prevalence of AS is stable, but since DISH may become more widespread due to its association with age, obesity and type 2 diabetes mellitus, a systematic review of the literature was conducted to increase the current knowledge on treatment, neurological status and complications of patients with preexisting ankylosed spines sustaining spinal trauma. A literature search was performed to obtain all relevant articles concerning the outcome of patients with AS or DISH admitted with spinal fractures. Predefined parameters were extracted from the papers and pooled to study the effect of treatment on neurological status and complications. Ninety-three articles were included, representing 345 AS patients and 55 DISH patients. Most fractures were localized in the cervical spine and resulted from low energy impact. Delayed diagnosis often occurred due to patient and doctor related factors. On admission 67.2% of the AS patients and 40.0% of the DISH patients demonstrated neurologic deficits, while secondary neurological deterioration occurred frequently. Surgical or nonoperative treatment did not alter the neurological prospective for most patients. The complication rate was 51.1% in AS patients and 32.7% in DISH patients. The overall mortality within 3 months after injury was 17.7% in AS and 20.0% in DISH. This review suggests that the clinical outcome of patients with fractures in previously ankylosed spines, due to AS or DISH, is considerably worse compared to the general trauma population. Considering the potential increase in prevalence of DISH cases, this condition may render a new challenge for physicians treating spinal injuries.