Pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) remains challenging with some difficulties, although it has been a well-established procedure. Its current situation including indications, surgical techniques with perioperative management, early and late outcome, and risk factors for mortality and poor hemodynamic improvement are reviewed. With the recent advancement of PEA including perioperative management and the accumulation of experiences, early outcome has been improved with low mortality rates, which are 5-10 % in most or <5 % in experienced centers. The risk factors for mortality were high pulmonary vascular resistance before and immediately after surgery, poor preoperative exercise capacity (NYHA-class IV), and advanced age. Reperfusion lung injury and residual pulmonary hypertension remain problematic as the most serious complications. The latter occurs in cases with surgically inaccessible distal lesions. For them, more careful perioperative management using pharmacological agents in conjunction with skillful PEA is required, occasionally with prompt use of percutaneous cardiopulmonary support. Although there have been a few reports on the long-term outcome, it is also favorable with good survival and event-free rates, which are affected by residual pulmonary hypertension. The recurrence of CTEPH after PEA is extremely rare. Consequently, as the first-line treatment for CTEPH, PEA can be performed safely with hemodynamic improvement and favorable early and long-term outcomes, except for potentially high-risk patients with distal lesions, elevated pulmonary vascular resistance, poor exercise capacity, and advanced age. Recently advanced balloon pulmonary angioplasty might be a promising alternative for such difficult patients.