A 50-year-old woman was admitted to our hospital on April 4, 1992, because of progressive worsening of dry cough and exertional dyspnea. Moderate hypoxemia and obstructive ventilatory impairment were present. Her chest roentgenogram and CT films showed thickened bronchovascular bundles in the absence of significant parenchymal fibrosis. Transbronchial lung biopsy revealed the formation of noncaseous epithelioid cell granulomas. Pulmonary perfusion scintigraphy showed multiple perfusion defects predominantly in the upper lung fields. Pulmonary ventilation scintigraphy showed a normal pattern. After administration of prednisolone for 3 months, her chest roentgenogram and CT films demonstrated marked decrease of infiltrates, but there was no improvement of hypoxemia and perfusion defects on pulmonary perfusion scintigraphy. Pulmonary angiography revealed multiple stenoses and occlusions of segmental and subsegmental branches of pulmonary arteries. Long-term steroid treatment will be necessary for this rare form of pulmonary sarcoidosis to prevent the development of pulmonary hypertension.