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We report here the first case of a clinically diagnosed sarcoidosis-like reaction in metastatic renal cell carcinoma following treatment with immune checkpoint inhibitors. Since the diagnosis of small renal masses is often a challenge despite improvements in imaging modalities, renal tumor biopsy provides useful information regarding treatment decisions. However, there is no established treatment strategy when renal biopsy shows lymphoid tissue. A 63-year-old woman was referred to our department for the investigation of a small renal ma