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Background: The right-sided aortic arch (RAA) is an uncommon anatomical anomaly found in <0.1% of the adult population. In this article, we report a case of RAA anomaly with an aberrant left subclavian artery (ALSA) and Kommerell's diverticulum associated with aneurysmal dilation of the ascending aorta, left carotid artery (CCA) stenosis, and pancake kidney presented with a transient ischemic attack (TIA). To the best of our knowledge, this is the first case in the literature that discusses such associations, especially in a symptomatic patient with neurological rather than tracheaesophageal symptoms and in the absence of the steal phenomenon., Case Description: A 52-year-old male, with a history of recurrent multiple TIAs, presented immediately after the onset of blurred vision and left-sided weakness. The initial diagnostic cerebral angiogram revealed a left CCA stenosis of <30%, with normal posterior circulation vasculature. The diagnosis of RAA was made with computed tomography angiography (CTA) of the thoracic and abdominal aorta, which revealed Type 2 RAA, with ALSA, which had a bullous dilatation at its origin that suggests Kommerell's diverticulum. Another two findings on CTA were a persistent left-sided superior vena cava that ended in the coronary sinus and a single pelvic fused renal mass (Pancake kidney)., Conclusion: We presented an extremely rare case of RAA with ALSA associated with a group of extra rare anomalies. Understanding the anatomical variants of RAA and its characteristics is critical to improving the management and follow-up of patients with such anomalies., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Surgical Neurology International.)