Background: Sporadic renal hemangioblastoma (HB) is an extremely rare malignancy whose progression and prognosis are still unknown. Case presentation: We describe a rare case of bilateral sporadic renal HB in a 58-year-old male with hematuria and flank pain. The diagnosis of this tumor was confirmed by typical imaging finding and negative immunostaining for cytokeratin (CKAE1/AE3), CD10, CD117, and positive staining for CD31, CD34, Vimentin and Inhibin-A in the biopsy specimen. Major demolitive surgey was avoided and during the subsequent 3 years no change in tumour size nor metastasis were observed. Conclusions: Immunohistochemistry (IHC) was critical for the differential diagnosis of HB from other more common renal tumours, especially renal cell carcinoma. To the best of our knowledge, this is the first case report of a HB involving both kidneys.