Significant advances have been achieved over the past few decades regarding comprehension of the pathogenesis of pulmonary arterial hypertension (PAH). The development of new agents and use of existing drug therapies have targeted the underlying abnormalities and pathways leading to progression of PAH. Milrinone, a phosphodiesterase inhibitor, remains a therapeutic option. Unfortunately, intravenous administration of the drug in patients with PAH may be limited by systemic hypotension, especially in those already receiving prostanoid treatment. We describe a 42-year-old woman with acute decompensated idiopathic PAH who was given nebulized milrinone as a novel adjunctive therapy. She was acutely treated with intravenous treprostinil 2 ng/kg/minute and inhaled nitric oxide 20 ppm. However, increasing the treprostinil infusion rate or adding other therapies such as intravenous milrinone for acute symptomatic relief was limited by her hemodynamic instability, which required treatment with dobutamine, vasopressin, and epinephrine. Nebulized milrinone was added as salvage therapy for her acute PAH crisis. After 8 days of therapy, the patient's PAH symptoms improved without compromising her mean arterial pressure and heart rate. Nebulized milrinone in addition to inhaled nitric oxide and low-dose intravenous treprostinil may have played a major role in the acute management of her PAH crisis. Further studies are needed to assess the role of nebulized milrinone in patients with PAH.