Chronic thromboembolic disease, a rare complication of pulmonary embolism (PE), is caused by incomplete resolution of thrombotic material in the pulmonary vasculature. It can result in symptoms of shortness of breath, exercise intolerance, and measurable functional limitations on cardiopulmonary testing. In it’s most severe form, chronic thromboembolic pulmonary hypertension (CTEPH) can lead to pulmonary hypertension (PH), right heart failure, and death. CTEPH can be difficult to diagnose and requires a high level of suspicion and appropriate screening tests. Once the diagnosis is made, a multidisciplinary evaluation to assess surgical candidacy for pulmonary thromboendarterectomy (PTE) should be performed. For inoperable patients, or those with residual or recurrent pulmonary hypertension after pulmonary endarterectomy, medical therapy and balloon pulmonary angioplasty are good alternative options.