A patient with cystic dystrophy in heterotopic pancreatic tissue, localized in the second duodenum, is described. Diagnosis was assessed by computed tomography, endosonography and magnetic nuclear resonance (MNR). Medical treatment with octreotid during three weeks was effective but duodenal stenosis persisted. Finally, pylorus preserving pancreaticoduodenectomy was performed. Microscopy examination confirmed the diagnosis and the efficiency of medical treatment. A review of the literature about cystic dystrophy is done.