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Following Kasai portoenterostomy for biliary atresia (BA), long-term survival in children is associated with chronic liver cirrhosis leading to hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH). This further causes a diverse array of respiratory and circulatory symptoms. The incidence of these complications in children with BA is higher than that seen in other chronic liver diseases. With a faster progression, BA is life-threatening in some severe cases. Moreover, HPS and PoPH may coexist in the same child. Enhancing our understanding of HPS/PoPH, making an early diagnosis, rational selection of medication, and appropriate timing of liver transplantation underscore the need for multidisciplinary collaboration.