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Objective To estimate the excess risk of cardiovascular and cerebrovascular diseases among individuals with ankylosing spondylitis (AS) in Quebec compared with the general population of Quebec. Methods A retrospective cohort study was conducted using population-based administrative data from Quebec. The cohort included all adult individuals with at least 1 AS diagnosis on physician billing or hospital discharge records between 1996 and 2006. A comparison cohort was generated using a 1% random sample of individuals without AS. Cardiovascular and cerebrovascular diseases, and associated hospitalizations, were classified into 1 of 6 subcategories: congestive heart failure, valvular (aortic or nonaortic) heart disease, ischemic heart disease, cerebrovascular disease, or “other” cardiovascular disease. The age- and sex-stratified prevalence estimates, and standardized prevalence ratios, of cardiovascular or cerebrovascular disease in patients with AS, compared to that in the general population, were calculated. Results The AS cohort included 8,616 individuals diagnosed over the period 1996–2006. The prevalence of cardiovascular and cerebrovascular diseases increased with increasing age for all cardiovascular disease subgroups, and was similar for individuals of both sexes. Age- and sex-stratified prevalence ratios were highest in younger individuals with AS. The age- and sex-standardized prevalence ratios comparing the risk among those with AS to the risk in the general population were as follows: for aortic valvular heart disease 1.58 (95% confidence interval [95% CI] 1.31–1.91), for nonaortic valvular heart disease 1.58 (95% CI 1.43–1.74), for ischemic heart disease 1.37 (95% CI 1.31–1.44), for congestive heart failure 1.34 (95% CI 1.26–1.42), for “other” cardiovascular disease 1.36 (95% CI 1.29–1.44), for cerebrovascular disease 1.25 (95% CI 1.15–1.35), and for any hospitalization for a cardiovascular or cerebrovascular disease 1.31 (95% CI 1.22–1.41). Conclusion Compared with the general population, patients with AS are at increased risk for many types of cardiovascular and cerebrovascular diseases, and are more likely to be hospitalized for these diseases. The excess risk is greatest in younger patients with AS.