Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHTN) are distinct clinical entities that may complicate liver disease. Although HPS and PPHTN are different, several reports describe 6 patients in whom both conditions have occurred, either concurrently or sequentially, sometimes with the onset of PPHTN after liver transplantation. The current report extends this sparse experience by reporting 2 patients who underwent liver transplantation for HPS and who developed pulmonary hypertension after liver transplantation. This experience calls for better understanding of the pathogenesis of HPS and PPHTN and ways to better predict their occurrence. Liver Transpl 12:1278-1282, 2006. © 2006 AASLD.