Cystic dystrophy of heterotopic pancreas (CDHP) is a rare condition. The cystic tissue is virtually always located in the duodenal wall, causing digestive tract obstruction and pain. Six cases in young males (mean age 40 years) managed since 1989 are reported. The diagnosis was established by computed tomography and/or endoscopic ultrasonography. All six patients underwent resection of the lesion, which required cephalic pancreatectomy in five cases. There were no death. The symptoms resolved in five patients; one alcoholic patient, had persistent pain. The diagnosis of CDHP can always be established preoperatively. Surgical excision is the best treatment.