We report a case of racemose hemangioma of the right bronchial artery complicated with an atrial septal defect (ASD) and ground-glass opacity localized in the right upper lung lobe. A 42-year-old woman was admitted for the pre-operative assessment of ASD. Aortography and selective angiography revealed that the right bronchial artery was markedly dilated and convoluted, draining into the right intercostal arteries and the right upper pulmonary vein. The patient was suffering from exertional dyspnea, but produced no bloody sputum. Since videobronchoscopy showed normal mucosal findings without bronchiectasis, primary racemose hemangioma of the right bronchial artery was diagnosed, and was treated by ligation of the bronchial artery and patch-closure of the ASD. The ground-glass opacity observed preoperatively in the periphery of the right upper lung lobe vanished after surgery. This opacity was considered to have been localized pulmonary congestion due to augmented hydrostatic pressure in the right upper pulmonary vein, induced by increased pulmonary blood flow through the ASD and the drainage route of the racemose bronchial artery into right upper pulmonary vein.