The purpose of this study is to present the characteristic HRCT findings of the lung parenchyma in patients with proximal interruption of the right main pulmonary artery. HRCT findings of proximal interruption of the right pulmonary artery demonstrated reticular opacities, septal thickening, subpleural consolidation, cystic lung changes, and pleural thickening in all 5 patients; bronchial dilation and bronchial wall thickening in 4 patients; and subpleural ground glass opacity (GGO) in 3 patients. The changes may be caused by absent pulmonary artery perfusion and development of systemic vessel collateralization.