Primary pulmonary hypertension continues to present both diagnostic and therapeutic challenges to the physician, reflecting the lack of understanding about the basic mechanism of the disease. The natural history of the condition has been described only recently, and this has allowed a reasonably accurate prognosis for the individual patient to be determined. New treatments have been found effective, including heart-lung transplantation, which can now offer improved survival. Long-term intravenous infusion of prostacyclin has a place in the treatment of severely affected patients awaiting heart-lung transplantation. However, the major impact of prostacyclin has been, perhaps, to concentrate research upon the role of the endothelial cell in the development of this disorder, in particular the potential role of an impairment of release of the recently described endothelium-derived relaxing factor in certain forms of pulmonary hypertension. We have attempted in this review to focus on the problems of diagnosis and treatment together with current developments in the understanding of primary pulmonary hypertension.