This is a case report of pulmonary sarcoidosis presenting with acute respiratory failure. A 33-year-old black man presented with 4 days of headache and fever. He had subsequent development of respiratory failure on the seventh hospital day. Workup showed high serum angiotensin-converting enzyme and increased pulmonary parenchymal uptake of gallium. Extensive workup for collagen vascular disease and infectious pathology were negative. Transbronchial biopsy specimens of the lung parenchyma showed noncaseating epithelioid granulomas consistent with sarcoidosis. The patient was treated with intravenous methylprednisolone succinate with dramatic clinical improvement.