Notably, treatment options for VHL disease have recently broadened with the introduction of belzutifan, a hypoxia-inducible factor 2α (HIF-2α) inhibitor approved by the Food and Drug Administration (FDA) in August 2021 for the treatment of VHL-associated RCC, CNS-Hb, and pNET that do not require immediate surgery [11, 12]. Here, VHL is linked to von Hippel-Lindau disease.