Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by calcification and ossification of the soft tissues, mainly ligaments and entheses. The spines of patients with DISH generally become increasingly rigid and osteoporotic, and fractures may occur after even a relatively minor traumatic event such as a ground-level fall. Moreover, the prevalence of DISH may be rapidly increasing in affluent societies. Thus, awareness of this condition is becoming more important for neurosurgeons when assessing trauma patients. For the present article, a literature review was conducted to summarize the current clinical, pathogenetic, and therapeutic knowledge of this disease. Furthermore, current treatment strategies for DISH-related spine injuries are also reviewed. Although the recommended treatment for spinal injuries in DISH patients is surgical, mainly through long-segment posterior fusion, rather than conservative options, stable fractures without any associated neurologic deficits have often been successfully managed with immobilization alone. Percutaneous instrumentation and the use of teriparatide may be useful depending on the surgical risks and patient neurological status.