A chest bruit and evidence of pulmonary hypertension developed in a woman with sarcoidosis. A perfusion lung scan revealed decreased perfusion to the right upper lobe and to the entire left lung, while a ventilation scan was normal. Pulmonary angiography demonstrated multiple bilateral concentric pulmonary artery stenoses most consistent with extrinsic compression by mediastinal granulomatous inflammation and fibrosis. The patient was treated with systemic corticosteroids for one year without improvement. Major pulmonary artery stenosis should be considered in patients with sarcoidosis who developed pulmonary hypertension and are found to have lung scans showing segmental perfusion defects in normally ventilated areas or who develop an unexplained chest bruit.