BACKGROUND: Vascular rings are a classical cause of tracheal and esophagus compression. We report the case of such an abnormality in an infant with neurofibromatosis. CASE REPORT: A 1 week-old male infant with a familial neurofibromatosis presented a stridor with severe respiratory distress. A vascular ring was demonstrated and operated on. The stridor persisted after surgery. A postoperative oesophagogram and tracheobronchoscopy showed an irregular compression of the oesophageal lumen, thought to be due to a residual extrinsic compression. Because the postoperative echocardiogram showed an extensive tumoral infiltration of both auricles, it was decided to not operate again the child. The postmortem examination revealed a disseminated neurofibromatosis infiltrating trachea, bronchi and also the wall of esophagus. CONCLUSION: Persisting stridor and oesotracheal compression postoperatively requires search for another cause. Association of vascular ring and neurofibromatosis is probably not fortuitous.