BACKGROUND: Earlier studies on stroke in celiac disease (CD) have been underpowered, but a recent study suggested that childhood CD is associated with a 10-fold increased risk of death from stroke, although it was based on small numbers. We examined the risk of stroke in patients with biopsy-verified CD. METHODS: We collected biopsy data from all 28 pathology departments in Sweden and identified 28,676 individuals with CD diagnosed between 1969 and 2007 (Marsh 3: villous atrophy). In the main analyses, we used Cox regression to estimate hazard ratios (HRs) for stroke in patients with CD compared with HRs for stroke in 141,806 sex- and age-matched controls. RESULTS: During follow-up, there were 785 first-stroke diagnoses in patients with CD and 2937 in reference individuals. Patients with CD were at increased risk of stroke (HR 1.10; 95% confidence interval [CI] 1.01-1.19). HRs were similar for ischemic stroke and brain hemorrhage and were not affected by adjustment for type 1 diabetes, rheumatoid arthritis, use of medication against hypertension, or dyslipidemia. The absolute risk of stroke in patients with CD was 267 per 100,000 person-years (excess risk 24/100,000). The highest risk estimates occurred in the first year, with virtually no increased risk after more than 5 years of follow-up after CD diagnosis. The HR for stroke in childhood CD was 1.10 (95% CI 0.37-3.22). CONCLUSIONS: Patients with CD are at only a small increased risk of stroke, which persists only for a brief period after diagnosis. CD does not seem to be a major risk factor for stroke.