Pulmonary vein varix is a rare abnormality, often resembling a pulmonary or mediastinal mass on chest radiographs, and pulmonary angiography has been the mainstay of diagnosis. An unusual case of pulmonary vein varix was clearly defined by echocardiography performed in a 47-year-old woman with chest discomfort who had been found to have an abnormal structure behind the cardiac silhouette on a chest radiograph. The diagnosis of left lower pulmonary vein varix was noninvasively confirmed by transthoracic echocardiography and transesophageal echocardiography.