Background-Studies on ischemic heart disease (IHD) incidence in individuals with celiac disease (CD) are contradictory and do not take small intestinal pathology into account. Methods and Results-In this Swedish population-based cohort study, we examined the risk of IHD in patients with CD based on small intestinal histopathology. We defined IHD as death or incident disease in myocardial infarction or angina pectoris in Swedish national registers. In 2006 to 2008, we collected duodenal/jejunal biopsy data on CD (equal to villous atrophy; Marsh 3; n = 28 190 unique individuals) and inflammation without villous atrophy (Marsh 1 to 2; n = 12 598) from all 28 pathology departments in Sweden. A third cohort consisted of 3658 individuals with normal mucosa but positive CD serology (Marsh 0, latent CD). We found an increased risk of incident IHD in patients undergoing small intestinal biopsy that was independent of small intestinal histopathology (CD: hazard ratio [HR], 1.19; 95% confidence interval [CI], 1.11 to 1.28; 991 events; inflammation: HR, 1.28; 95% CI, 1.19 to 1.39; 809 events; and latent CD: HR, 1.14; 95% CI, 0.87 to 1.50; 62 events). Celiac disease (HR, 1.22; 95% CI, 1.06 to 1.40) and inflammation (HR, 1.32; 95% CI, 1.14 to 1.52) were both associated with death resulting from IHD, whereas latent CD was not (HR, 0.71; 95% CI, 0.34 to 1.50). Conclusions-Individuals with CD or small intestinal inflammation are at increased risk of incident IHD. We were unable to show a positive association between latent CD and incident IHD.