Chronic thromboembolic pulmonary hypertension (CTEPH) is responsible for significant levels of morbidity and mortality. The estimated cumulative incidence of CTEPH is 2–4% among patients presenting with acute pulmonary thromboembolism. Currently, at the time of CTEPH diagnosis, 37.9% of the patients in an international registry were receiving at least one pulmonary arterial hypertension (PAH)-targeted therapy. Advanced medical therapy is considered in patients with inoperable disease, as a bridge to pulmonary endarterectomy or in those with persistent or recurrent pulmonary hypertension. PAH-specific medical therapies include endothelin receptor antagonists, phosphodiesterase inhibitors, and prostacyclin analogues. The present article will focus on recent developments in the pharmacological treatment of CTEPH.