The present perspective is a synthesis of 80 published investigations in the setting of a retroaortic left brachiocephalic vein, described in 250 patients. Clinical presentation, radiographic findings, ultrasonographic findings, saline contrast echocardiography, computed-tomographic angiocardiography, magnetic resonance imaging, and angiocardiography provided the diagnostic information used to define the disease entity prior to considering the surgical approach to the associated cardiac anomalies. We have also addressed several issues concerning the influence of isomerism, the establishment of diagnosis, and its importance in various surgical and interventional procedures. Although the retroaortic left brachiocephalic vein is asymptomatic, its recognition during clinical investigation should raise the possibility of an association with other malformations, especially right aortic arch, ventricular septal defect, and anomalies of the outflow tracts. We submit that an increased appreciation of this venous anomaly may facilitate surgical planning, endovascular procedures, placement of central venous lines, and transvenous pacemakers.