About 5–10% of pancreatic cancers have been linked to an underlying genetic disorder, usually inherited in an autosomal dominant manner. We have previously studied the risk of cancer in patients with cystic fibrosis and found that, although the overall risk of cancer was not increased, there was a fivefold increase in the risk of digestive tract cancer.^1,2 Because only four patients developed pancreatic cancer, it was difficult to estimate the risk accurately. Since the publication of these reports we have obtained information on additional patients with both cystic fibrosis and pancreatic cancer from the US cystic fibrosis patient registry, from published reports, and by querying surgeons, pathologists, and cystic fibrosis physicians in the USA, Canada, and Europe.

We now describe nine patients with cystic fibrosis and pancreatic cancer identified during the study period (1985 to 2006), including …