Primary pulmonary hypertension is a progressive disease with a mean survival of < 3 years from the time of diagnosis. Recent advances in the medical management have shown that some patients may have an extremely good outcome depending on the response to high doses of calcium antagonists. Anticoagulants are also associated with improved survival, and prostacyclin is showing great promise in patients who are refractory to conventional therapy. At the same time, lung transplantation has developed into a viable treatment option for patients who remain symptomatic and deteriorate on medical management. The role of the various medical treatment modalities, and the ideal timing of lung transplantation, is an issue that is continuing to evolve as improvements in all treatments develop. The cost and availability of the various treatments will likely affect the selection of treatments in individual patients as well.