Interrupted pulmonary artery is a very rare congenital abnormality. It may be detected at an early age or in adulthood. It may be associated with congenital cardiovascular defects. However, it is usually isolated and detected incidentally in adults. In this article, we present clinical and radiological findings of a 50-year-old female patient with interrupted right pulmonary artery and stenosis of the inferior pulmonary veins in the affected lung, in light of the literature review.