Background: The most common aortic arch abnormality is an aberrant right subclavian artery (ARSA). ARSA-esophageal fistula is a rare sequela that can present with a life-threatening upper gastrointestinal (GI) bleed. Case Report: We report the case of an 88-year-old male who presented with signs of upper GI bleeding. Esophagogastroduodenoscopy demonstrated extrinsic compression of the upper third of the esophagus with ulceration. Imaging studies revealed ARSA posterior to the esophagus with pseudoaneurysm formation. These findings confirmed an upper GI bleed secondary to ARSA-esophageal fistula. The patient underwent prompt embolization of the ARSA pseudoaneurysm, followed a few days later by coil embolization of the ARSA pseudoaneurysm. Despite these interventions, the patient continued to have bleeding with anemia. He and his family opted to avoid any further interventions and instead pursued comfort care. The patient was discharged to hospice and died 3 months later. Conclusion: ARSA-esophageal fistula is a rare but potentially lethal cause of upper GI bleeding. Initial signs and symptoms can be subtle, but the presence of a GI bleed requires immediate stabilization. Surgical interventions have been shown to have longer-lasting success, but endovascular repair may be an option for patients who are deemed unfit for surgery but still require prompt stabilization. Regardless of the intervention, mortality rates for ARSA-esophageal fistula are high.