Endothelin-1 (ET-1) is known to be a principal factor in the pathogenesis of primary pulmonary hypertension (PPH). Recently intravenous administration of epoprosterol improved the survival rate in PPH. However, the effect of epoprosterol on ET-1 remains to be investigated. Therefore, we studied a patient with PPH who was treated with a low dose of epoprosterol and examined the serum concentration of ET-1 during the treatment. Epoprosterol greatly decreased the serum concentration of ET-1 in parallel with improvement of the clinical course, suggesting that ET-1 level may be a marker for treatment of PPH.