Abnormalities of the aortic arch occur mainly due to an abnormal development of the fourth aortic arch in the first 12 weeks of fetal life. These abnormalities may be asymptomatic for a long period, or can develop clinically when vascular rings surround the trachea and esophagus and cause tracheal and/or esophageal obstruction. We present the case of a full-term newborn baby, male. The pregnancy had no medical surveillance from a family physician or an obstetrical. After five days of age, the infant died due to severe malformations, incompatible with life, respectively due to single arterial trunk and aorta coarctation. In the literature, there have been cited several other cases similar to our case; however, this one remains an extremely rare anomaly. We believe that the abnormality is due to a regression on an abnormal site of the fourth left aortic arch, but due to the very small number of these anomalies reported so far, we cannot say with certainty if there is an embryologic reason for these abnormalities.
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