We present the case of a 67-year-old woman who developed a metastatic adenocarcinoma of the pancreatobiliary system shortly after the histologically confirmed diagnosis of autoimmune pancreatitis (AIP). This case highlights the need for increased alertness not only in differentiating AIP from pancreatic cancer at the time of diagnosis, but also to exclude concomitant malignancies of the pancreatobiliary system in the management of histologically confirmed AIP.