Annular pancreas (AP) is a rare congenital anomaly. Coexisting malignancy has been reported only in a few cases. We report what is, to the best of our knowledge, the first case in the English literature of duodenal adenocarcinoma in a patient with AP. In a 55-year old woman with duodenal outlet stenosis magnetic resonance cholangiopancreatography showed an aberrant pancreatic duct encircling the duodenum. Duodenojejunostomy was performed. Eight weeks later she presented with painless jaundice. Duodenopancreatectomy revealed a duodenal adenocarcinoma, surrounded by an incomplete AP. Thus, co-existent malignancy with AP can be present without obstructive jaundice and without being visible through preoperative diagnostics.