Necrotizing sarcoid granulomatosis (NSG) is a rare granulomatous pneumonitis which is composed of a background of sarcoidosis-like granulomas, granulomatous vasculitis, and variable amount of necrosis. We reported a case of a 38-year-old nonsmoking woman presented with left-sided chest pain and dyspnea for three days. Chest CT scan exhibited collapse consolidation of the left lower lobe with the presence of two separated small-sized cystic lesions within the collapsed segment. Lung lesion resection was performed, and histological examination confirmed the diagnosis by excluding other causes of granulomatous diseases. The prognosis of NSG is favorable, and medical treatment is usually not necessary, as well as in our case. NSG is a rare disease with nonspecific symptoms and good prognosis which is frequently confused with Wegener's granulomatosis, sarcoidosis, and Churg-Strauss syndrome. This entity should also be considered as differential diagnosis of necrotizing granulomatous diseases.