Pulmonary vascular diseases encompass a large and diverse group of underlying pathologies ranging from venous thromboembolism to congenital malformations to inflammatory vasculitides. As a result, patients can present either acutely with dyspnea and chest pain or chronically with dyspnea on exertion, hypoxia, and right heart failure. Imaging, particularly with multidetector CT, plays a key role in the evaluation and management of patients with suspected pulmonary vascular disease and, given the widespread routine use of high-quality CT pulmonary angiography, it is imperative that radiologists be familiar these pathologies.