Chronic thrombembolic pulmonary hypertension (CTEPH) represents a common type of pulmonary hypertension and is associated with significant morbidity and mortality. The prognosis of this condition reflects the degree of associated right ventricular dysfunction. Unresolved thrombemboli and possible in situ thrombosis lead to obstruction of pulmonary arteries resulting in elevated pressures in those areas of the vasculature that were spared from thromboembolic occlusion. This can resemble pathomechanisms in patients with pulmonary arterial hypertension. However the understanding of pulmonary vascular remodeling in patients with CTEPH is incomplete. Pulmonary endarterectomy of the obstructing thromboembolic material should be performed in patients who are accessible to surgery. In patients who are judged inoperable or with persistent pulmonary hypertension treatment with pulmonary arterial hypertension medications can be considered. This could contribute to improved clinical outcome and survival, whereas further controlled studies are required addressing this question.