目的探讨von Hippel-Lindau病(VHL病)的影像学特征。资料与方法回顾性分析7例VHL病影像学表现以及临床、病理资料。结果7例VHL病影像学表现包括均有1处或1处以上的中枢神经系统血管母细胞瘤,5例有1处或多处包括胰腺多发囊肿、双肾多发肿瘤和囊肿以及阴囊肿瘤在内的内脏病变。结论VHL病为家族性遗传性多系统肿瘤综合征,熟悉其影像学表现有利于正确诊断。 Objective To investigate the imaging features of von Hippel-Lindau disease (VHL). Materials and Methods Retrospective analysis of 7 cases of VHL disease imaging and clinical and pathological data. Results The imaging manifestations of 7 patients with VHL included one or more central nervous system hemangioblastoma, one with multiple or multiple pancreatic cysts, multiple nephridial neoplasms and cysts and scrotal tumors in 5 cases Visceral lesions. Conclusion VHL is a familial hereditary multi-system tumor syndrome. Familiar with the imaging findings is conducive to the correct diagnosis.