Pleural effusion, diffuse endobronchial mucosal involvement and distal airway obstruction were observed in a 50-year-old woman. Chest X-ray revealed mediastinal lymphadenopathy and diffuse parenchymal infiltrates. Extensive extrapulmonary involvement was not present. Both transbronchial lung biopsy and pleural biopsy demonstrated non-caseating granulomas consistent with sarcoidosis. The patient was given prednisone which was continued for 18 months in gradually decreasing doses. This treatment resulted in marked improvement of bronchoscopic findings. The simultaneous presence of pleural effusion and endobronchial involvement in the same patient is a rare occurrence in sarcoidosis.