We describe a case of 29 year old man, a first Polish patient with idiopathic arterial hypertension (IPAH) listed from Poland and successfully treated with lung transplantation in Vienna. Time from diagnosis to lung transplant was merely 11 months. Rapid clinical deterioration required treatment with most of currently approved or emerging methods, including oral and parenteral prostacyclin analogues administration by inhalation and chronic subcutaneous infusion. Atrial balloon septostomy was used to bridge the patient to transplant. We describe multiple problems in providing pharmacotherapy and in arranging logistics for lung transplantation. Peri- and multiple post-transplantation complications including dehiscence of right main bronchial anastomosis and its successful therapy are also presented. We consider good long term outcome as assessed 26 months post transplantation as an encouragement for other attempts at lung transplantation in patients with IPAH and for development of this method of therapy in Poland.