A 3-year-old boy was admitted to the local hospital due to a lack of appetite and fatigue for over a month. Pulmonary arterial hypertension (PAH) was found a half month ago. He was diagnosed with idiopathic pulmonary arterial hypertension (IPAH) after other causes were ruled out. Oral sildenafil and bosentan were used to control IPAH. He was transferred to our hospital. Cardiac catheterization indicated severe PAH (pulmonary artery pressure/systemic circulation pressure = 0.81) and right heart failure. During hospitalization, the child repeatedly suffered from pulmonary hypertension crises. He was treated with intravenous milrinone and treprostinil, but pulmonary artery pressure (PAP) did not significantly decrease. After multidisciplinary consultation, he was scheduled for a Potts shunt surgery, in which the left pulmonary artery was anastomosed to the descending aorta. Perioperative management of PAH was key to the success of Potts shunt surgery.