This chapter summarizes current knowledge about chronic thromboembolic pulmonary hypertension (CTEPH), and discusses the perioperative and anesthetic management of patients scheduled for pulmonary endarterectomy (PEA). It discusses the pathophysiology, prognosis, diagnosis, and treatment for CTEPH. Approximately half of the patients exhibit known thrombotic tendencies like protein-C deficiency, factor V Leiden deficiency, anti-phospholipid syndrome or other autoimmune disorders. Computerized tomography scanning with pulmonary angiography is a useful tool in distinguishing between proximal and distal disease. The peri-operative management of patients undergoing pulmonary endarterectomy is complex and involves a multidisciplinary team of physicians, surgeons, anesthetists and intensivists. Thorough preoperative investigation and ascertaining the appropriateness of surgery is essential. Generally quoted and accepted peri-operative mortality risk is 10%, down to 4% in centers with large experience. In most surviving patients, a marked reduction in the pulmonary arterial pressure is observed and right heart function will return to normal within a few months.