We would like to emphasize that the possibility of a PAX8-positive intracranial HB does exist and must be taken into account by neuropathologists to avoid misdiagnoses; in this regard, a broader immunohistochemical panel also including CD10, Inhibin-α, PAX2, S100, and anti-Renal cell carcinoma (RCC) antibody is highly recommended. The gene discussed is PAX2; the disease is hereditary clear cell renal cell carcinoma.