Society of Diabetic Nephropathy Prevention Journal of Nephropathology 2251-8363 15 1 2026 01 01 Sporadic hemangioblastoma of the kidney; a rare entity and a potential diagnostic challenge e26569 e26569 10.34172/jnp.2025.26569 EN Shirin Taraz Jamshidi https://orcid.org/0000-0002-5954-493X Salman Soltani https://orcid.org/0000-0002-2135-6757 Hamidreza Ghorbani https://orcid.org/0000-0001-9814-3277 Tina Zeraati https://orcid.org/0000-0001-6250-830X Naser Tayyebimeibodi https://orcid.org/0000-0003-2114-4671 Atena Aghaee https://orcid.org/0000-0002-1351-3142 Ali Emadzadeh https://orcid.org/0009-0000-7311-1164 Journal Article 10.34172/jnp.2025.26569 2024 08 24 2025 09 19 Hemangioblastoma is a benign tumor of central nervous system (CNS) which may rarely occur in extraneural tissues. Renal hemangioblastoma (RH) occurs sporadic or in the setting of Von-hipple-lindau syndrome. It is usually misdiagnosed as other renal masses including renal cell carcinoma (RCC) due to their similar clinical and histologic features. In this paper, we present two cases of RH which resembled RCC, however immunohistochemical studies confirmed diagnosis of RH.