Ankylosing spondylitis (AS) is a chronic inflammatory joint disease that can lead to chronic pain in axial and peripheral joints and to functional impairments after several years. Excess mortality has been reported in patients with AS. We reviewed recent studies of patients with AS who were treated and monitored according to the improved methods developed in the past few years, without radiation therapy. Our results do not support excess mortality in these patients. Long-term follow-up data from patients enrolled in biologics registries will provide additional information. Cardiovascular disease is the leading cause of death in patients with AS, as in the general population. However, the cardiovascular mortality rate may be slightly increased in patients with AS, probably as a result of dyslipidemia and early endothelial dysfunction. Similarly, and as expected, there is excess mortality related to the spinal disease itself and to renal and gastrointestinal disease. More surprisingly, alcohol abuse and injury or suicide cause excess mortality compared to the general population. In the absence of radiation or radium-224 therapy, and regardless of the other treatments used, the evidence does not support an increased rate of lymphoma or other malignancies compared to the general population. In this review, we discuss the causes and rates of mortality in patients with AS.