VHL病是一种家族性常染色体显性遗传性肿瘤病,病变可累及多个器官、表现多样,包括中枢神经系统血管母细胞瘤、内脏肿瘤和内脏多发囊肿等。对本病的了解将有助于对本病的及时诊断,避免误诊和漏诊该病。本文将报道1例并发双侧肾癌的VHL患者并结合文献阐述VHL病的临床特点、诊断方法以及治疗。 VHL disease is a familial autosomal dominant hereditary neoplasm disease. Lesions can involve multiple organs and perform various functions, including central nervous system hemangioblastoma, visceral tumors and visceral multiple cysts. The understanding of this disease will help to timely diagnosis of the disease, to avoid misdiagnosis and missed diagnosis of the disease. This article will report one case of VHL patients with bilateral renal cell carcinoma combined with literature to describe the clinical features of VHL disease, diagnosis and treatment.