The clinical, functional, radiologic, and pathologic characteristics of seven cases of necrotizing sarcoid granulomatosis (NSG) are presented. The population consisted of five women and two men, with an average age of 36 years. Each patient's predominant presenting complaint was pleuritic chest pain. Pulmonary function testing demonstrated a variety of abnormal patterns. Computed tomography (CT) of the chest showed solitary or multiple nodules in all patients, occasionally associated with pulmonary infiltrates in the lower lobes. Pleural involvement was seen on CT scanning in six patients and mediastinal adenopathy was present in five. Biopsy specimens of the lung lesions revealed confluent epithelioid granulomata associated with necrosis and vasculitis. Pleural involvement by confluent granulomata was a prominent feature in four patients. Follow-up has ranged from 6 months to 4 years. All patients are now asymptomatic, the majority having received prednisone. One patient received methotrexate as a steroid-sparing measure. We conclude that NSG is distinguishable from sarcoidosis as a clinicopathologic entity in which pleural involvement is a frequent finding. Treatment with steroids appears to hasten recovery.