BACKGROUND: The association between inflammatory bowel disease (IBD) and eosinophilic esophagitis (EoE) remains unclear. We aimed to perform a systematic review and meta-analysis on the association between EoE, IBD, and other immune-mediated inflammatory diseases. METHODS: A systematic literature search was conducted in Ovid MEDLINE, Ovid Embase, and the Cochrane Central Register of Controlled Trials (Ovid) from inception to July 2023. The primary outcome was to compare the frequency of an EoE diagnosis in IBD and other immune-mediated inflammatory disease processes versus control populations. Where pooled analysis was possible, we reported odds ratios (ORs) with 95% confidence intervals (CIs). I2 values were used to report heterogeneity, with values >50% suggesting substantial heterogeneity. RESULTS: We identified 2612 eligible studies, of which 38 studies were included. A diagnosis of EoE was significantly greater in patients with IBD compared to the general population (OR 3.9; 95% CI, 2.6-5.9 [I2 = 99.5%]). There was no significant increase in EoE in patients with ulcerative colitis compared to Crohn's disease (OR 1.0; 95% CI, 0.7-1.3 [I2 = 88.7%]). EoE was also significantly increased in patients with atopic dermatitis (OR 2.4; 95% CI, 1.9-3.1 3 [I2 = 70.4%]) compared to those without atopic dermatitis. CONCLUSIONS: Both IBD and atopic dermatitis were associated with an increased odds of EoE diagnosis. Further research is needed to determine the underlying mechanisms behind these potential associations.