Von Hippel-Lindau disease (VHL) is a major heritable renal cell cancer (RCC) syndrome. 24-45% of VHL patients have a cumulative risk to develop RCC. The management of VHL has changed significantly as clinicians have learned how to best balance the risk of cancer dissemination while minimizing renal morbidity. Despite the improving management of this disease, there are no evidence-based guidelines of the treatment of localized and advanced RCC in VHL. Here, we describe a 39-year-old patient with VHL disease who developed metastatic RCC and received sunitinib with complete remission.