Von Hipple-Lindau病(VHL)是中枢神经系统少见的多发性真性血管性肿瘤,国内很少报告,现将我科收治1例综合文献报告如下: 患者 女性,38岁。1986年初,发现左眼视物不清,经北京同仁医院眼科诊为视网膜血管畸形。后又逐渐出现双上肢麻木无力。同年行脊髓碘水造影及头颅CT报告“未发现异常”。1989年出现双下肢无力, Von Hipple-Lindau disease (VHL) is a rare multiple true vasculature of the central nervous system. It is rarely reported in China. The comprehensive literature of one case in our department is reported as follows: Patients Female, 38 years old. In early 1986, the left eye was found to be unclear, and was diagnosed as a retinal vascular malformation by the Beijing Tongren Hospital. Then gradually appeared numbness in both upper extremities. In the same year, the spinal cord iodine radiography and head CT report “no abnormalities were found.” In 1989, there was weakness in both lower limbs.