Introduction: Duodenal atresia and stenozis represants a congenital malformation of the duodenum. Duodenal obstruction may be complete or incomplete. Duodenal atresia is an example of complete intrinsic obstruction, characterized by lack of a duodenal segment or it's transformation to a fibre cord, without structure and lumen, and clinical appears as a neonatal occlusive syndrome. Duodenal stenosis is an example of an incomplete intrinsic abnormality. The incomplete nature of the obstruction in duodenal stenosis results in a variable and often delayed presentation till the age of 10-15 years. Case presentation: A 6 weeks old femaile patient presents after 12 hours of birth with respiratory symptoms: marked nasal obstruction, abundant secretions in the posterior pharynx, tachypnea, intercostal tiraje, pulmonary with transmission rales, laryngeal stridor, rhythmic heart sounds, with systolic murmur grade III/6, bilious vomiting, multiple genetic stigmas: little an appropriated eyelid slots, retrognathia, simian crease, multiple hemangiomas. Management and findings: Plain and contrast radiography confirms the diagnosis of duodenal stenosis by revealing the classic double-bubble sign, representing air in the stomach and proximal duodenum, which is associated with complete or near-complete duodenal obstruction. The definitive management is surgical correction: duodeno-entero-anastomosis is performed. Conclusions: Postoperative evolution is good, without early postoperative complications, with a favorable prognosis, due to the lack of multiple associated anomalies and an extreme prematurity. [ABSTRACT FROM AUTHOR]