The treatment of newly diagnosed metastatic renal cell carcinoma (mRCC) evolved dramatically with the approval of immune checkpoint inhibitors (ICI) such as nivolumab, ipilimumab, and pembrolizumab for this indication. Herein, we describe the case of a 52-year old male patient, without chronic diseases and with a 30-pack-year smoking history, who was diagnosed with mRCC (clear cell carcinoma) including enlarged lymph nodes in the mediastinum, a mass in the pleura, and numerous metastases in both lungs. The patient was treated with a combination of nivolumab and ipilimumab, followed by nivolumab monotherapy, which is still ongoing (as of December 2021). The patient had a near-complete response (near resolution of the metastatic lesions) and did not experience adverse events. After 13 months of treatment, and in light of the near-complete response, the patient underwent a radical laparoscopic nephrectomy. The postoperative period was uneventful and the patient was discharged from the hospital 3 days after surgery. Examining the excised kidney revealed no residual tumor, connective tissue, signs of inflammation and necrosis. As of December 2021 (approximately 23 months from immunotherapy initiation) the patient had no evidence of disease. This case report demonstrates a treatment approach involving deferred nephrectomy after (and during) ICI treatment. The response of the patient described herein to a combination of nivolumab and ipilimumab is consistent with the available data supporting the efficacy of this combination as a first-line therapy in mRCC. Currently, the evidence supporting deferred nephrectomy (after ICI) vs upfront nephrectomy and then ICI, or ICI alone without nephrectomy is limited to a few retrospective studies. Thus, prospective randomized studies are needed to elucidate the role of deferred nephrectomy in mRCC. Two phase 3 studies (PROBE and NORDIC-SUN) that were designed to address this issue are currently enrolling patients and their results are expected within several years.