Snap Shot A 5-year-old child presents with an occipital headache, an ataxic gait, nystagmus, and papilledema. CT shows a cranial hemangioma. Introduction Autosomal dominant Associated with deletion of VHL gene (tumor suppressor) on chromosome 3. Leads to increased risk of: multiple hemagiomas in various organs hemangioblastomas renal cell carcinoma pheochromocytoma Presentation Mental status changes Headache Gait change Hematuria