Arterial tortuosity syndrome is a rare connective tissue disorder characterized by elongation and tortuosity of large elastic arteries resulting in multiple vascular stenoses. This article reports on a novel hybrid transcatheter-surgical strategy to treat bilateral peripheral pulmonary artery stenoses due to this syndrome. Operation consisted of stent implantation by midline sternotomy and reductive reconstruction of the proximal pulmonary arteries. This approach resulted in right ventricular overload relief and sudden improvement of the patient's clinical condition.