Granulomatous inflammation with multinucleated giant cells is observed in various infectious and noninfectious diseases. It has been found in association with malignant tumors and designated sarcoid-like reaction. The distinction between a tumor-related granulomatous reaction and a true sarcoidosis can be a problematic issue. A case of renal cell carcinoma with sarcomatoid features (Fuhrman nuclear grade IV) with an extensive peritumoral sarcoid-like reaction and a critical review of the few cases of this association described in the literature have been reported, and the problematic clinical and pathological assessments of such lesions are discussed.