(UroToday.com) Von Hippel-Lind (VHL) syndrome occurs because of hereditary inactivating genomic alterations in the VHL gene, resulting in a higher risk of developing renal cell carcinoma, retinal and CNS hemangioblastomas, and other tumors. Management of VHL syndrome-associated kidney neoplasms is challenging as tumors continue to arise despite surgical removal, which can result in renal insufficiency. To balance the risk of metastasis with renal morbidity, tumors are often observed until they reach 3 cm in size, at which point they are resected. Additional management strategies are required to manage VHL associated RCC.