Chronic thromboembolic pulmonary hypertension is a condition that is recognised in an increased percentage of patients. Pulmonary endarterectomy is recognised as being the only curative option for a subgroup of those patients, but anaesthesiologists and intensivists face many challenges in how they manage these patients perioperatively. Ultimately, it is the combination of skills in a multidisciplinary team that leads to a successful procedure and dramatically improves patient's quality of life and life expectancy.