Abstract: Objective To study the onset characteristics,diagnosis and treatment of von Hippel-Lindau（VHL） disease accompanying bilateral renal carcinoma. Methods Clinical data about 3 patients with VHL disease accompanying bilateral renal carcinoma admitted to our hospital from February 2007 to June 2011 were retrospectively analyzed with the related literature reviewed. Results One of the 3 patients had a family history and underwent laparoscopic radical resection of carcinoma in the left kidney with the renal unit of the right kidney preserved.Pathological examination suggested that it should be a clear cell carcinoma and hemodialysis should be performed for renal transplantation.The other two patients had multiple pathological lesions,including bilateral renal carcinoma accompanying multiple renal cysts,and underwent selective bilateral nephron-sparing and renal cyst surgery.Pathological examination showed that it was a clear cell carcinoma with normal renal function. Conclusion VHL disease with unique clinical features is different from sporadic renal cancer,its diagnosis mainly relies on MRI and CT,and operation is the main procedure for it.It is easy to relapse with many organs involved,thus needing comprehensive and multidisciplinary treatment.