Immunotherapies targeting programmed death-1 (PD-1)/programmed death- ligand 1 (PD-L1) and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4), otherwise known as immune checkpoint inhibitors (ICIs), have revolutionized the therapeutic landscape of genitourinary malignancies such as renal cell carcinoma and urothelial carcinoma. This evidence concerns the gene CTLA4 and hereditary clear cell renal cell carcinoma.