Chronic thromboembolic pulmonary hypertension (CTEPH) is an important differential diagnosis in patients with unexplained dyspnoea. CTEPH is under-recognized and carries a poor prognosis without treatment. Surgical pulmonary endarterectomy is the preferred treatment for the majority of patients. Advances in surgical and anaesthetic techniques and post-operative intensive treatment have reduced perioperative morbidity and mortality. Pulmonary endarterectomy results in major improvement of haemodynamics and clinical status and offers excellent long-term survival. It is most often a curative treatment. The surgical treatment of CTEPH in Denmark is centralized at Aarhus University Hospital. Pulmonary vasodilators and pulmonary balloon angioplasty are supplementary treatment options in this patient group.