We report the case of a young female with disphagia and weigth loss caused by a vascular ring associated with right aortic arch, Kommerell diverticula, and left retroesophageal ligamentum arteriosum (ductus arteriosus). The patient underwent surgical treatment. A left thoracotomy was performed. Surgical technique included diverticulum ressection and an aortopexia. There were no major complications. We also discuss the incidence, pathology, diagnosis, clinical features and treatment of this rare disease.