Pulmonary artery aneurysm (PAA) is a rare condition that results from several pathologies. Here, we report a case of a rare giant PAA accompanied by left lung compression due to recurrent chest tightness for >13 years. Through this case and review of the literature, we explore the treatment options to improve the diagnosis and treatment of PAA. We found that patients with PAA may survive longer even if they also have pulmonary hypertension. In addition, it is recommended that if a PAA diameter is >5.5 cm or if the PAA diameter has increased by >0.5 cm in 6 months, the PAA should be operated on as soon as possible. Conservative treatment can be recommended in patients with congenital PAA if pulmonary arterial pressure is normal.