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BACKGROUND: Inflammatory bowel disease (IBD) is a chronic inflammatory immune-mediated disorder of the gut with frequent extra-intestinal complications. Pancreatic involvement in IBD is not uncommon and comprises a heterogeneous group of conditions, including acute pancreatitis (AP), chronic pancreatitis (CP), autoimmune pancreatitis (AIP) and pancreatic exocrine insufficiency (PEI); however, data on such an association remain sparse and heterogeneous. METHOD: PubMed/MEDLINE and EMBASE databases were searched for studies investigating pancreatic involvement in patients with IBD. RESULTS: Four thousand one hundred and twenty-one records were identified and 547 screened; finally, 124 studies were included in the review. AP is the most frequent pancreatic manifestation in IBD; the majority of AP cases in IBD are due to gallstones and drugs but cases of idiopathic AP are increasingly reported. AIP is a rare disease, but a strong association with IBD has been demonstrated, especially for type 2 and ulcerative colitis. The pathogenetic link between IBD and AIP remains unclear, but an immune-mediated pathway seems plausible. An association between CP and PEI with IBD has also been suggested, but data are to date scarce and conflicting. CONCLUSION: This is the first systematic review of the association between IBD and pancreatic diseases. Gallstones and drugs should be considered the most probable causes of AP in IBD, with type 2 AIP also being possible. Systematic review. Pancreatic involvement in inflammatory bowel disease (IBD). The wide spectrum of pancreatic involvement in patients with IBD may represent a challenge. From the 124 studies analyzed, acute pancreatitis (AP) is the most frequent pancreatic manifestation in IBD; the majority of AP cases in IBD are due to gallstones and drugs, but cases of idiopathic AP are increasingly reported. Autoimmune pancreatisis is a rare disease, but a strong association with IBD has been demonstrated, especially for type 2 and ulcerative colitis.[Image: see text]