Anesthetic care for patients undergoing pulmonary endarterectomy represents one of the most challenging tasks in cardiac anesthesia. Chronic thromboembolic pulmonary hypertension with its concomitant right ventricular failure may cause hemodynamic instability during anesthetic induction and the precardiopulmonary bypass (CPB) period, and the associated comorbidities (pulmonary, hepatic) may affect the actions and metabolism of anesthetic drugs. During the CPB period, proper perfusion patterns, cerebral oxygenation, and adequate hypothermia for deep hypothermic circulatory arrest must be achieved. During the post-CPB period the anesthesiologist must be prepared to treat residual pulmonary hypertension, pulmonary edema, pulmonary bleeding, right ventricular failure, and various metabolic and cardiovascular sequelae of hypothermic circulatory arrest. This review highlights the main issues the anesthesiologist faces during pulmonary endarterectomy, as well as suggests approaches to their management.