Myxomas are the most common benign primary cardiac tumors in adults, occurring in 95% of the cases in the atria, typically in the left atrium.They very rarely present outside the heart, and their incidence is currently unknown due to the few reported cases.We present the case of a 53-year-old man who was admitted with chronic ischemia of the left upper limb, due to a dependent lesion in the aorta.Imaging studies led to a presumptive diagnosis of myxoma of the aortic arch, and the patient underwent tumor resection.The diagnosis was confirmed by histopathology.