A 16-month-old girl with a diagnosis of severe valvular pulmonary stenosis, ventricular septal defect and unilateral absence of the right pulmonary artery proven by cardiac catheterization, angiography and surgery is presented. The right lung was smaller than the left one on chest X-ray, and aortography showed that it was supplied by a rudimentary circulation arising from intercostal branches of the descending thoracic aorta.
