Autoimmune pancreatitis (AIP) occurring in association with inflammatory bowel disease (IBD) is rather rare and carries a worse prognosis and greater disease severity compared with IBD alone. Although it is an infrequently documented association, progress over the last 20 years has led to better understanding of the association between AIP and IBD. IBD has a stronger association with type 2 than with type 1 AIP. Clinical and histologic features of AIP-IBD more often reveal features of type 2 AIP. Imaging is not helpful in facilitating the diagnosis of AIP and IBD. Similarly, attempts to identify a serological marker have not yielded better result. A proposed lymphocyte homing mechanism provides some insight into the mechanism of pathogenesis. This review represents an update of our current knowledge of the association between AIP and IBD.