Pulmonary artery hypertension (PAH) is a rare but lethal disease that affects the pulmonary vascular bed, resulting in hypoxia, respiratory distress, right heart failure, exercise limitation and mortality. Currently, many PAH specific medications are applied to ameliorate patients’ symptoms, improve life quality and prolong their lives. The survival rate has improved with medical therapy but patients may still suffer from insufficient exercise capacity. Therefore, cardiopulmonary exercise test (CPET) can play an important role in the evaluation of PAH patients’ risk status and treatment response, and, furthermore, it can guide the rehabilitation program. In this article, we would like to introduce the current implementation of CPET and rehabilitation for PAH patients.