Objective To Analyze of the clinical data of the same family two patients with VHL syndrome to improve the level of diagnosis and treatment. Methods Retrospective analysis of the two cases of VHL syn⁃drome patients was done for clinical characteristics, including imaging findings, and relevant organ disease. Results Both patients underwent cerebral aneurysms surgery. One with routine left kidney cancer was radically resected, the right renal multiple kidney cancers were resected and the right renal multiple cysts were operated to open the cyst wall with a pathologic report of transparent renal cell carcinoma. The second patient received conservative symptomatic treatment since in this patient the cancer affected many organs, not indicated to sur⁃gery. Conclusion VHL syndrome is a familial autosomal dominant genetic disease, it changes the perfor⁃mance of the central nervous system vascular mother cell tumor with celiac multiple visceral tumor, visceral cyst; comprehensive imaging examination and reasonable surgery are helpful to diagnosis treatment, and impor⁃tant means of follow-up.% 目的：分析同一家系2名Von Hippel-Lindau综合征(VHL综合征)患者的临床资料，提高VHL综合征的诊治水平.方法：回顾性分析我院收治的2例VHL综合征患者的临床特点，包括影像学表现，及相关脏器病变情况，探讨本病的诊断和治疗方法.结果：2例患者均行脑血管瘤手术，例1行左肾癌根治、右肾多发肾癌切除、右肾多发囊肿去顶术，术后病理肾透明细胞性肾细胞癌；例2肿瘤累及多器官，无法手术，行保守对症治疗.2例均行观察随访.结论：VHL综合征是一种家族性常染色体显性遗传病，病变表现为中枢神经系统血管母细胞瘤合并腹腔多发脏器肿瘤，脏器囊肿，全面的影像学检查和合理的手术是诊断、治疗、随访的重要手段.
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