Twelve cases of necrotizing sarcoid granulomatosis are presented as a retrospective study. The population of patients consisted of ten women and two men, with an average age of 50 years. Nine patients initially had a variety of pulmonary and nonpulmonary complaints, while three were asymptomatic. Chest roentgenograms demonstrated bilateral nodules in seven patients, solitary nodules in four, and a miliary pattern progressing to nodules in one. Enlarged hilar nodes were found in six. Histologically, all biopsies showed a combination of individual granulomas, confluent masses of granulomas which formed the nodular masses seen on the roentgenograms, a variable amount of necrosis of tissue and hyalinization, and a granulomatous vasculitis. Follow-up periods ranged from four months to 11 years. Eleven patients are alive and asymptomatic. The sole death occurred in a patient treated with an immunosuppressive agent (cyclophosphamide). We conclude that the clinical behavior of necrotizing sarcoid granulomatosis is not similar to that of the other angiocentric granulomatoses and that most patients with this disease can be left untreated or be treated with steroids alone. We suggest the possibility that necrotizing sarcoid granulomatosis may be the histologic counterpart of so-called nodular sarcoid.