Surgery is a key part of treatment strategies for Von Hippel-Lindau syndrome (VHL), with surgical techniques often being used to control the metastatic potential of tumors. However, there are limitations with this treatment strategy, as there are only so many times that an organ can be operated on. Dr Ramaprasad Srinivasan, MD, PhD, National Cancer Institute, Bethesda, MD, discusses the novel treatments that have been investigated for patients with VHL over the past few decades. He explains the rationale and development process of the investigation of belzutifan, as well as clinical trial results which have led to FDA approval of belzutifan in some patients with VHL. This interview took place at the American Society of Clinical Oncology (ASCO) 2022 Annual Meeting in Chicago, IL.