Inhaled nitric oxide (NO) has been reported to promote selective pulmonary vasodilation and better arterial oxygenation in cases of adult respiratory distress syndrome (ARDS) with pulmonary hypertension (PHT). However, the clinical relevance of these changes and their effects on outcome remain to be proven, since long-term inhalation carries a potential risk of toxicity. We used NO to treat a patient who developed postpneumonectomy ARDS with severe PHT and right ventricular failure. NO resulted in better oxygenation and markedly improved hemodynamic status. As the underlying disease progressively worsened the patient became fully dependent on NO for hemodynamic support, and he died after 24 days of effective supportive therapy with inhaled NO.