(UroToday.com) Von-Hippel Lindau (VHL) disease is an autosomal dominant hereditary disorder, which harbors an increased risk of developing renal cell carcinoma (RCC), CNS, and retinal hemangioblastomas, pancreatic neuroendocrine tumors, and other lesions. RCC occurs in 25-60% of individuals and is a key cause of morbidity and mortality. Surgery is generally considered when the largest renal tumor reaches approximately 3 cm. The goal of surgery is to decrease the risk of metastases. Unfortunately, tumors will continue to recur, and repeated surgeries are required to control other VHL disease manifestations. Despite aggressive treatment, life expectancy is shortened in patients with VHL.