A newborn presenting duodenal stenosis by small bowel malrotation and annular pancreas is reported. Major diagnostic problems in duodenal obstruction of newborn are considered in keeping with literature review and personal experience. In this presentation clinical appearance has been long aspecific (apnea and bradycardia spells) in spite of a rather typical radiological picture. Really, the true diagnostic nature of midgut obstruction is often intraoperative. Face to any duodenal obstruction in a newborn the possibility of many malformations of the gut should be raised and consequently looked for accurately by the surgeon for a good postoperative prognosis. The surgical procedure of choice is frequently a duodeno-jejunostomy or a duodeno-duodenostomy; the annular pancreas should, on the contrary, never be sectioned.