Vascular rings are congenital anomalies of the aortic arch system characterized by a vascular anomalies of mediastinal vessels that encircle and compress the esophagus and trachea. Aortic arch anomalies may be associated with other congenital cardiac defects or chromosomal anomalies, such as microdeletion of chromosome 22q11. In neonatal/ pediatric life, clinical presentation shows stridor and difficulty in swallowing or dysphagia. Case Report: We describe a particular case diagnosed in fetal

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... fe with a symptomatic vascular ring in neonatal life: left circumflex retroesophageal aortic arch, right patent ductus arteriosus "PDA"/ligamentum arteriosum, and aberrant right subclavian artery "ARSA." Conclusion: To the best of our knowledge, we described a novel vascular ring diagnosed in fetal life and symptomatic in neonatal life. In this extremely rare case, a "true" vascular ring, like in case of double aortic arch, it is due by the left circumflex retroesophageal aortic arch, ARSA, and right patent ductus/ligamentum arteriosum.