Ankylosing spondylitis (AS) is a chronic inflammatory rheumatic disease which causes pain along with loss of physical function and quality of life over many years. It has also been shown to increase mortality compared to the general population, independent of treatment modalities. Cardiovascular deaths are increased, and recent studies suggest both an abnormality of lipid regulation and microvascular changes. Increased rates of suicide, accidental death, and alcohol-related deaths have also been reported. This review examines rates and causes of increased mortality in AS and highlights a need to focus on cardiovascular risk factors and psychological health in addition to physical disability in patients with AS.