Therapeutic strategies for isolated unilateral absence of a proximal pulmonary artery remain unclear. The natural history of the disease, or thrombosis of primary surgical anastomosis, leads to exclusion of the affected lung with increased risk of intrapulmonary bleeding, impaired quality of life, and shortened life expectancy. We herein describe our two-stage approach in a small series of patients starting with interventional catheterization followed by surgical anastomosis. Other medical interventions, such as anticoagulation and pulmonary vasodilatation, are key factors to successfully restore pulmonary circulation in this rare defect.