Snapshot A 33-year-old man presents to the emergency department after noticing blood in his urine. Medical history is notable for a recently diagnosed left eye retinal hemangioblastoma, for which ophthalmology is following. Family history is notable for his father having some sort of brain and pancreatic cancer. A CT abdomen demonstrates a left-sided renal mass with areas of necrosis and calcifications. Introduction Definition login to view 6 more bullets Epidemiology Incidence login to view 1 more bullet etiology Pathophysiology login to view 3 more bullets Presentation Symptoms/physical exam login to view 24 more bullets Imaging MRI brain and spinal cord with and without gadolinium login to view 2 more bullets Abdominal CT or MRI login to view 2 more bullets Studies VHL molecular genetic testing login to view 2 more bullets Differential Isolated malignancies login to view 2 more bullets Treatment Multidisciplinary approach to manage benign and malignant tumors login to view 1 more bullet Complications Blindness from retinal hemangioblastomas Renal failure in patients with renal lesions Myocardial infarction or stroke in patients with pheochromocytomas Intestinal or biliary compression in pancreatic lesions