Rare disease Background: Pulmonary artery aneurysm (PAA) is a rare disease in cardiovascular system. This disease is difficult to diagnose and less often considered due to its non-specific clinical manifestations. Until now there are no clear guidelines about its optimal management because of the small number of reported cases. Case Report: We report a 56-year-old male with chief complain of atypical bilateral chest pain and shortness of breath. Initial electrocardiogram (ECG) and laboratory evaluation showed no sign of ischemic heart disease. After the patient was stabilized, he was evaluated using chest x-ray, transthoracic echocardiography (TTE), and multi slice computed tomography (MSCT). The patient was then diagnosed with PAA secondary to suspicion of pulmonary hypertension (PH) with chronic obstructive pulmonary disease and heart failure. Conservative treatment was chosen because of the limited resources for surgery and patient's refusal to be referred. The treatment aims to lower the pulmonary artery pressure while monitoring the aneurysm. His 6-month follow-up evaluation showed an improvement in pulmonary artery pressure and persistent of the PAA without any increasement of the diameter. Conclusions: PAA is a rare disease that is difficult to diagnose because of its non-specific nature. Persistent atypical chest pain can be an early symptom of PAA, thus clinicans should be aware in a high-risk patient suffered persistent chest pain, despite normal ECG and laboratory findings. TTE and MSCT evaluation are reliable for diagnosing PH and PAA. With conservative treatment and routine follow-up, patient with PAA secondary to PH could be managed well.