Renal cell carcinoma (RCC) is a largely chemotherapy-resistant disease that is commonly treated with molecularly targeted therapies. Evidence suggests that RCC is also an immune-responsive disease, and checkpoint inhibitors are in active development as agents for the treatment of systemic disease. Programmed cell death 1 (PD-1) is an inhibitory immune checkpoint, and blockade of the PD-1 cascade is an attractive target in RCC. Expression of the ligand for PD-1 in RCC has been shown to be a negative prognostic factor; however, response to PD-1 blockade is not restricted to tumors expressing the ligand for PD-1. Nivolumab is the most completely characterized anti-PD-1 agent in RCC and has been shown to be efficacious as monotherapy. Currently, there are multiple ongoing clinical trials exploring the use of combination therapy with PD-1 blockade.