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A vascular ring formed by a left aortic arch, a retro-esophageal segment, a right descending aorta and right-sided ductus or ligamentum arteriosum is a very rare anomaly up to now; only 16 cases have been reported in the literature. This report presents the case of an infant suffering from the symptoms of airway and esophageal compression, who was successfully operated on for this anomaly at the age of 5 months. The diagnosis was established by means of esophagogram and angiography. Diagnostic criteria and guidelines for therapy are discussed.
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