Objective      To explore the clinical and imaging characteristics of von Hipple-Lindau (VHL) syndrome

in order to improve the level of recognition and diagnosis of the disease.       Methods      The clinical and imaging data of 8 patients with VHL syndrome confirmed by imaging and pathology were retrospectively analyzed

which was combined with literature review.       Results      Eight VHL syndrome patients performed 64-layer spiral CT and 3.0T magnetic resonance imaging scanning and enhanced scanning

and five patients had family history of illness. There were 8 cases with central nervous system vascular tumor (frequent in 3 cases)

3 cases with retinal vascular tumor

6 cases with renal clear cell carcinoma and (or) renal cyst

1 case with renal angiomyolipoma

4 cases with pancreas multiple cysts

2 cases with pheochromocytoma

2 cases with liver vascular tumor and (or) cyst (frequent in 1 case)

and 1 case with liver abscess and lung abscess. Among them

4 cases were diagnosed as VHL syndrome-IA type

2 cases diagnosed as VHL syndrome-IB type

and 2 cases diagnosed as VHL syndrome-IIB type.       Conclusion      VHL syndrome often shows multiple organ involvement and poor prognosis. Imaging examination is helpful to qualitative diagnosis

disease screening and long-term follow-up for the members of the family.