EMANGIOBLASTOMA (HB), a kind of benign tumor with uncertain histogenesis, is characterized by the presence of stromal cells (SCs) and a rich vascular component. 1 It occurs sporadically, except for about 25% of the cases associated with von Hippel-Lindau (VHL) disease. HB typically occurs in the cerebellum, but it has been reported that HB occasionally occurs in extraneural tissues, such as kidney, 2 adrenal, 3 gastrointestinal tract, 4 soft tissue 5 , and so on. We reported an atypical case involving the kidney, which might be misdiagnosed for other renal tumors, especially clear cell renal cell carcinoma (RCC). We also reviewed previously published cases and literature, and made necessary molecular genetic study, in order to investigate its clinicopatholoical features and differential diagnosis, etc.