The current guidelines recommend the use of immune check inhibitors (anti–PD-1/anti–PD-L1 and anti-CTLA4) alone or in combination with anti–vascular endothelial growth factor (anti-VEGF) therapy in the first-line treatment of renal cell carcinoma, based on a substantial survival benefit as compared with anti-VEGF therapy alone. The gene discussed is CTLA4; the disease is hereditary clear cell renal cell carcinoma.