A 55-year-old man with schistosomal liver disease presented with shortness of breath, orthodeoxia, platypnea, cyanosis, marked digital clubbing and liver failure. Extensive investigation revealed no other etiology for liver disease apart from schistosomiases. The diagnosis of hepatopulmonary syndrome was based on clinical grounds, as well as abnormal arterial blood gases and positive contrast echocardiography. The patient underwent orthotopic liver transplantation, which was initially successful, but then died of respiratory complications and multi-organ failure on day 42 post-transplant. To the authors' knowledge this is the first report of hepatopulmonary syndrome associated with schistosomal liver disease.