Objective To investigate the clinicopathological features of central nervous system hemangioblastoma in von Hippel-Lindau syndrome.Methods The data of 10 patients with central nervous system hemangioblastoma in our hospital since 2013 were analyzed retro-spectively,and the clinicopathological features of central nervous system hemangioblastoma in von Hippel-Lindau syndrome were summarized. Results The macroexamination result showed that most tumor lesions were found in the cerebellum and medulla oblongata,with cystic chan-ges,size from 1 cm to 5 cm,the average size was (3.1 ±0.2)cm,clear boundary,intracapsular yellow cyst fluid.The microscopy result showed tumor foci with a rich blood supply,endothelial cell proliferation and hypertrophy in vascular,which arranged in nests or lobulated mesenchymal cells,the cytoplasm of stromal cells was abundant and lightly stained,a rich lipid was seen,with vesicular or foam.Conclusion The von Hippel-Lindau syndrome is usually cystic lesion,the microscopic examination shows tumor foci with rich blood supply,endothelial cell proliferation,hypertrophy and other changes in vascular.