Chronic thromboembolic pulmonary hypertension is a commonly overlooked cause of physical incapacity and dyspnoea, with a higher incidence than is generally appreciated and a poor prognosis. The diagnosis can be suspected based on echocardiographic examinations and ventilation perfusion scanning of the lung. Pulmonary angiography still remains the gold standard for the diagnosis of thromboembolic pulmonary hypertension and the assessment of operability. Pulmonary endarterectomy is a complex surgical procedure, which provides permanent relief of thromboembolic pulmonary hypertension and cure for most of the patients. The operation resembles a true endarterectomy of the pulmonary artery branches using extracorporeal circulation and periods of hypothermic circulatory arrest. In experienced centres, the operative risk has been decreased to an acceptable level. Following successful surgery, long-term survival and quality of life are excellent. Earlier referral to surgery might avoid the occurrence of a secondary vasculopathy in the unaffected areas of the pulmonary vascular tree, and therefore further improve early and late results. A multidisciplinary approach and surgical experience are the prerequisites for success of this demanding procedure.