Cystic dystrophy in heterotopic pancreas (CDHP) is characterized by the presence of cystic formations surrounded by inflammation and scarring. It usually involves the duodenal wall and can be responsible for strictures and pain. The diagnosis of this disorder was previously based on pancreatoduodenectomy specimens removed for a suspected pancreatic tumor. Six cases were observed in young men (mean age 40 years) between 1989 and 1993. Computed tomography (CT) and endoscopic ultrasonography (EUS) features allowed definitive preoperative diagnosis of CDHP. After surgical resection of the tissue-bearing segments that included five pancreatoduodenectomies and one antrectomy, symptoms disappeared in all patients. Patients were followed 2 to 45 months; one patient experienced recurrence of pain and hyperamylasemia 17 months after surgery. The preoperative diagnosis of CDHP is presently possible because of modern imaging procedures and improved knowledge of specific signs. Resection is the most appropriate treatment.