Because of its clear cell morphology, the abundant vascular component and its association with vHL mutations and/or VHL syndrome, intracranial HBs must be frequently distinguished by metastatic ccRCCs; accordingly, additional immunohistochemical antibodies including PAX8, CD10 and RCC9,10 are often required to avoid a misdiagnosis that would be extremely deleterious for the patient as HBs are low-grade and slow-growing lesions, successfully treated with surgical resection, while surgery plus adjuvant radiotherapy are required for metastatic ccRCC. The gene discussed is PAX8; the disease is von Hippel-Lindau disease.