Congenital vascular anomalies of the thorax represent an important group of entities that can occur either in isolation or in association with different forms of congenital heart disease. It is extremely important that radiologists have a clear understanding of these entities, their imaging characteristics, and their clinical relevance. The imaging armamentarium available to diagnose these diverse conditions is ample, and has evolved from such traditional methods as chest radiography, barium esophagography, and angiography to new modalities that include echocardiography, multidetector row CT (MDCT), and MR imaging. These imaging modalities have added safety, speed, and superb resolution in diagnosis and, as in the case of MDCT, provide additional information about the airway and lung parenchyma, resulting in a more comprehensive examination with greater anatomic coverage. This article reviews the most important congenital thoracic vascular anomalies, their embryologic foundation, clinical presentation, and imaging characteristics, especially those of MDCT.