Belzutifan (MK-6482, previously called PT2977), a highly specific and well-tolerated HIF-2α inhibitor, received approval from the US Food and Drug Administration (FDA) on August 13, 2021 for the treatment of VHL disease patients requiring therapy for associated nonmetastatic RCC, pNETs, and CNS hemangioblastomas and carrying VHL germline mutations (26, 44, 45). This evidence concerns the gene VHL and von Hippel-Lindau disease.