Chronic thromboembolic pulmonary hypertension is a rare, severe and continuous deterioration of pulmonary vascular disease which has high mortality rate along with disease progression. Earlier diagnosis could contribute to a more accurate treatment plan. A case of 67-year-old female was admitted to hospital because of exertional dyspnea. She was initially suffering from heart failure with acute deterioration. After admission, the results of medical history inquiry, laboratory data and physical examination revealed that BNP level kept raising and the electrocardiogram showed S1Q3T3, which pulmonary embolism with heart failure was suspected. Therefore, echocardiography revealed pulmonary hypertension (pulmonary artery pressure: 62.3 mmHg) and right heart failure. Pulmonary embolism was noticed on chest computed tomography. Soon after the family meeting of medical condition discussion, the patient received right heart catheterization combined with pulmonary artery angiography, and the final diagnosed is distal chronic thromboembolic pulmonary hypertension with right heart failure. After medical team’s assessment, the treatment plan was based on armaceutical control. Thus, the target therapy of Riociguat was performed. For such type of patients, even with non-specific symptoms, the possibility of pulmonary embolism can still be predicted based on medical history, laboratory data, physical examination, and the imaging differential diagnosis. If diagnosis and treatment can be conducted as early as possible, the high mortality rate of chronic hromboembolic pulmonary hypertension can be reduced.