Objective　To explore the clinical characteristics of pulmonary arterial hypertension associated with congenital portosystemic shunt (PAH-CPS) in children. Method　The clinical data of PAH-CPS in children admitted from January 2012 to January 2019 were retrospectively analyzed. Results　In 5 children (2 males and 3 females)， the age at initial diagnosis was from 0.08 to 4.4 years. The main manifestations of 5 patients were recurrent pulmonary infection, hepatosplenomegaly, abnormal liver function and pulmonary arterial hypertension. Echocardiography confirmed that all the children had different degrees of pulmonary arterial hypertension and intracardiac malformations. Abdominal ultrasonography showed intrahepatic and extrahepatic portosystemic shunts. Abdominal contrast-enhanced CT and cardiac catheterization showed the formation of intrahepatic and extrahepatic vessels. Conclusions　PAH-CPS can be manifested as repeated pulmonary infection, hepatosplenomegaly, abnormal liver function, etc. Ultrasonography can provide diagnostic clues. Abdominal enhanced CT scan and cardiac catheterization can make a definite diagnosis.