Abstract Purpose Annular pancreas encountered in adults and jejunal arterial variations are rare. Anatomical variations can cause conflicts between oncology and surgical safety. Methods Case report of a 68-year-old man suffering from vomiting because of an annular pancreas and a ductal adenocarcinoma of the pancreas head invading the second portion of the duodenum. Results Contrast-enhanced computed tomography showed multiple arterial variations describing the absence of the coeliac trunk such that the left gastric artery (LGA), splenic artery and superior mesenteric artery (SMA) were arising separately from the aorta. The accessory left hepatic artery arose from the LGA; and both the common hepatic artery and combined trunk of the replaced right hepatic artery with the higher replaced first jejunal artery separately arose close to the root of the SMA. The patient underwent curative pancreaticoduodenectomy which achieved 3 years of recurrence-free survival. Conclusion This was an extraordinary case of annular pancreas with first jejunal arterial variation detailing an embryological interpretation as well as considerations for balancing short- and long-term outcomes.