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Necrotizing sarcoid granulomatosis (NSG) is a subgroup of idiopathic vasculitic syndromes, which is rare and usually has a limited role in the diagnosis of pathological findings. In the pathology, varying rates of necrosis, vasculitis and granulomas are seen. Here we are presenting a case of this rare disease. A 54-year-old female patient was referred to our clinic with a differential diagnosis of malignancy-metastasis-vasculitis according to the chest X-ray. Thorax computed tomography of the patient showed multiple smoothly limited nodular lesions at first glance suggesting metastasis and no mediastinal lymphadenopathy. P-ANCA positivity and total IgE elevation were detected in the patient with collagen tissue markers and serum ACE level in normal range. Tomography guided transthoracic tru-cut biopsy was performed. “Intact granuloma structures consisting of multinuclear giant cells and epithelioid histiocytes scattered on the inflamed ground, eosinophilic necrosis, and microabces foci with dominant focal neutrophil clusters were observed. In specific dyes, fungal and mycobacterial infections were excluded. Because of not fulfilling the diagnostic criteria, eosinophilic granuloma with polyanjitis (Churg-Strauss syndrome) was excluded. Although the role of literature in pathogenesis is not yet fully understood, p-ANCA positivity has been reported in sarcoidosis, even if there is no systemic vasculitis. Necrotizing sarcoid granulomatosis was diagnosed with clinical, radiological, pathological and laboratory findings. Because of the symptoms of the patient, steroid treatment revealed significant clinical and radiological improvement. The patient is still under control with 16 mg corticosteroid treatment.