Pulmonary hypertension is one of the indications for lung transplantation. Recent advances on medical management of the disease have dramatically decreased the number of patients who required lung transplantation. In reported series, single or double lung transplantation have been successfully undertaken by many transplant centers. In patients, whose cardiac functions are irreversibly damaged heart-lung transplantation remains as an only option for long term survival. Transplantation should only be considered in those patients who are having optimal medical support and relatively good condition for a major operation.