An anomalous inferior vena cava with azygos continuation is a distinct entity now being diagnosed more frequently in patients with congenital heart defects. As a congenital abnormality, it may become apparent at any age, but is certainly more often noticed in children undergoing cardiac catheterization. The incidence of this anomaly in patients with congenital heart defects has been stated to be 0.6 per cent, and in most clinically diagnosed cases, cyanosis is produced by the cardiac defects. Abnormal positions of the heart, such as mirror image dextrocardia, dextroversion, situs inversus with levocardia or levoversion, and partial inversion of the abdominal viscera (heterotaxia) are common, as well as additional anomalies of the superior vena cava. Most of the reported cases have been described from observations in the dissecting laboratory (2, 3) and usually designated “absence of the vena cava,” although, as Hollinshead states (4), neither the infrarenal portion nor the suprahepatic portion is apparently ever absent. The infrarenal portion, in fact, may be normally situated, but the inferior vena cava does not have its usual course. What should be the upper end of the inferior vena cava, as it pierces the diaphragm and enters the heart, is formed entirely by the hepatic veins and thus represents the venous return from the liver only. The anomalous vena cava, after receiving the renal veins, passes into the thorax through the aortic hiatus and becomes continuous with either the azygos or the hemiazygos vein. Usually, such cases are interpreted as being due to a failure of development of the anastomosis between the right subcardinal vein and the hepatic veins, with a persistence of the upper end of one or both supracardinal veins which in earlier stages drained upward to the superior caval system. Of special interest to the radiologist in this condition is the distinct configuration often seen in the dilated azygos vein, which sometimes presents as a mediastinal tumor. This tumefaction may produce a protuberance at the junction of the enlarged azygos vein with the superior vena cava. The largest single series clinically described to date is that of Anderson et al. (1). They encountered a total of 15 cases of absent inferior vena cava in approximately 2,500 new patients with congenital heart defects observed over a six-year period at the University of Minnesota Hospital and reviewed 26 cases previously published. These authors described a typical “candy cane” appearance of the anomalous venous pathway. When angiocardiography was performed from the leg vein, the most common pattern encountered was azygos continuation with normal position of heart and abdominal viscera. Other authors (5, 6) have also described isolated cases in which the dilated azygos vein simulated a mediastinal tumor. Occasionally, surgical intervention was undertaken.