The incidence of small bowel cancers is increasing. Associations have been made between celiac disease and small bowel cancers, but there have been no detailed studies of large cohorts.Through the nationwide ESPRESSO cohort study, we retrieved data from Sweden's 28 pathology departments on all individuals who received a diagnosis of celiac disease diagnosed from 1965 through 2017. Individuals with celiac disease, defined as duodenal or jejunal villous atrophy (stage 3 Marsh score) were matched with as many as 5 randomly selected reference individuals from the general population. We used stratified Cox regression to calculate hazard ratios (HRs) for small bowel adenocarcinoma, adenomas and carcinoids.During a median follow up of 11 years, we identified 48,119 individuals with celiac disease (patients) and 239,249 reference individuals. Beginning at 1 year after a diagnosis of celiac disease, 29 patients (0.06%) received a diagnosis of small bowel adenocarcinoma vs 45 reference individuals (0.02%), 7 patients received a diagnosis of carcinoids vs 31 reference individuals, and 48 patients received a diagnosis of adenomas vs 50 reference individuals. Corresponding HRs were small bowel adenocarcinoma 3.05 (95% CI, 1.86-4.99), carcinoids 0.59 (95% CI, 0.16-2.10), and adenomas 5.73 (95% CI, 3.70-8.88). HRs were independent of sex and age. Overall, there was 1 extra case of small bowel adenocarcinoma in every 2944 patients with celiac disease followed for 10 years. There was an inverse association between mucosal healing risk of future small bowel adenocarcinoma (HR, 0.18; 95% CI, 0.02-1.61), although the HR failed to attain statistical significance.In an analysis of a nationwide pathology database in Sweden, we found the absolute risk of small bowel adenocarcinoma is low in individuals with celiac disease. However, risks of small bowel adenocarcinoma and adenomas (but not carcinoids) are significantly increased in people with celiac disease compared to people without this disease.