HPS should be ruled out in a patient with hypoxemia and chronic hepatic disease and knowledge of such a syndrome is essential to formulate a diagnosis – thus influencing therapeutic strategies. Management should equally involve pulmonologists, hepatologists and, in particularly severe cases, transplant surgeons in a multidisciplinary approach. Patients with HPS are given a higher priority on the waiting list for liver transplantation based on the observation that liver transplantation outcome may be poorer in cases of advanced disease. Without liver transplantation the prognosis for HPS is poor with mortality usually because of complications of the hepatic disease rather than to a primary respiratory event.