In VHL-associated ccRCC, long-term results from LITESPARK-004 show a sustained response to Belzutifan in patients with VHL, where 67% (95% CI, 54–79%) of patients with renal tumors achieved an objective response, paving the way for a new treatment paradigm for these patients [28]. The gene discussed is VHL; the disease is nonpapillary renal cell carcinoma.