Von Hippel-Lindau (VHL) disease is a rare genetic condition than can lead to the development of various tumors and cysts in the body, with brain and spinal hemangioblastomas (benign tumors caused by abnormal blood vessel growth) being the most common. Early diagnosis through genetic testing and regular check-ups is important to prevent serious complications, such spontaneous hemorrhage. In this report we describe two cases of young patients who developed symptomatic brain hemangioblastoma much earlier than usual. We discuss how combining both genetic and clinical factors could help to improve early detection and treatment strategies for VHL disease. (By Vicente Santa-María López, https://jmg.bmj.com/content/early/2025/03/26/jmg-2024-110477 )