Objective To investigate the clinical features,diagnosis and treatment of von Hippel-Lindau (VHL) disease in order to improve the understanding of this disease.Methods The clinical features,imaging features,pathological data and treatments of 7 patients in 3 families admitted to our hospital from December 2005 to August 2017 were retrospectively analyzed.Results Ten oprations were performed under the guidance of neural navigation system for the 7 patients.There were 4 enjoying total resection at once;one achieved primary surgical resection of the primary lesions,and residual lesions grew up and the patient accepted surgical treatment at the 7th and 9th years;one patient achieved primary surgical resection of the primary lesion,the residual lesions grew up and surgical treatment was performed 8 months later;another one underwent surgical resection of the primary lesion,and the residual lesion was treated with gamma knife therapy 3 months after surgery.The pathological results of the 10 operations of the 7 patients were proved to be hemangioblastoma.No operative deaths were noted.The symptoms of these patients when they were discharged from the hospital were significantly better than those when they were on admission.The 10 patients were followed up so far,no symptoms of neurological impairment were noted,and they could work and live a normal life.Conclusions Surgical treatment is still the preferred method of VHL,and radiotherapy is effective for small lesions.