Pulmonary hypertension due to chronic thromboembolic disease is potentially curable with pulmonary thromboendarterectomy surgery. As a result, it is important for clinicians to recognize and appropriately diagnose this form of pulmonary hypertension. Advances in this field with changes in surgical technique, the availability of PH-targeted medical therapy for select patient subgroups, and the development of balloon pulmonary angioplasty have broadened therapeutic options for patients. This review will examine what is known about the epidemiology and medical conditions placing patients at risk of developing this disease, will present an approach to evaluation of patients with suspected chronic thromboembolic disease, and will describe the surgical and non-surgical management of this unique patient population.