Partial anomalous pulmonary venous connection, a rare congenital anomaly, most commonly involves the right lung, with one or more pulmonary veins anomalously connecting most frequently to the superior vena cava and less commonly to the right atrium or inferior vena cava. This article describes an unusual case of anomalous pulmonary venous connection of the right lung to the azygos vein in an adult. This anomaly was clearly delineated with angiography, computed tomography of the chest, and transesophageal echocardiography. The transesophageal echocardiographic features of the anomaly are described as a means to prevent further diagnostic misinterpretation.