Duodenal cystic dystrophy due to ectopic pancreas deposit is an uncommon pathology. Diagnosis is made by modern imaging techniques, mainly endoscopic ultrasound which localizes precisely cysts in duodenal wall. The most frequent clinical symptoms are pain, duodenal obstruction, and weight loss. We report the case of a 40 year-old man with cystic dystrophy of the 2nd part of the duodenum, without chronic pancreatitis, treated by a conservative surgical procedure including segmental duodenal resection. This original approach is an alternative to the Whipple procedure.