Renal cell carcinoma (RCC) is a highly vascular tumor, which may spread to the lungs and other organs. It often presents with localized or systemic manifestation, including paraneoplastic syndromes. Sarcoidosis is a systemic granulomatous inflammatory disease characterized by non-caseating granulomas that typically afflicts the respiratory system. In the absence of any evidence of systemic sarcoidosis they are referred to as sarcoid-like reactions. Non-caseating epithelioid granulomas, also regarded to sarcoid-like granulomas have been described in association with certain malignancies such as carcinomas of the breast, colon, seminoma, and Hodgkin's lymphoma. However, sarcoid like reaction associated with renal cell carcinoma is uncommon. Herein we present a rare case of a patient with renal cell carcinoma with mediastinal lymphadenopathy initially thought to metastatic disease, though revealed a sarcoid-like reaction with review of literature.