Necrotizing sarcoid granulomatosis (NSG) is an unusual pulmonary lesion first described by Liebow and thought to represent a variant of Wegener granulomatosis. In contrast to Wegener granulomatosis, NSG contains well-formed granulomas in a lymphangitic distribution suggesting its relationship to sarcoidosis. Also, NSG has a better prognosis than Wegener granulomatosis. The mean age of presentation is approximately 50 years, with a male-to-female predominance of 2:1. Presenting symptoms include cough, dyspnea, fever, and chest pain. Originally, the disease was thought to involve only the lungs; however, subsequent reports have demonstrated systemic disease with multiple organ involvement. Several reports suggest that NSG is a variant of nodular sarcoidosis. Grossly, the lesions of NSG are gray-white, well-circumscribed masses with central necrosis.