Ankylosing spondylitis (AS) is associated with accelerated atherosclerosis and enhanced cardiovascular morbidity and mortality compared to the general population. The mechanisms and mediators of this phenomenon have not been fully explained, but an expanding body of evidence demonstrates that increased cardiovascular risk in AS is heralded by endothelial dysfunction. We performed a literature review using the PubMed database from the year 2006 up to 2018. In this article we review the epidemiology, current evidence for impaired endothelial function, potential mechanisms and markers controlling this dysfunction, and finally we summarize the data regarding the efficacy of pharmacotherapy in reducing endothelial dysfunction in patients suffering from AS.