A 46-year-old woman presents with walk instability. A diagnosis of cerebellar hemangioblastoma is made on MRI and neurosurgical excision was performed. In the postoperative course, the patient died of a bulbar cerebral stroke with respiratory distress. At the autopsy, the finding of a bilateral renal clear cell carcinoma in addition to the cerebellar hemangioblastoma allows for the diagnosis of von Hippel-Lindau disease. It is an inherited, autosomal dominant syndrome charaterized by a VHL gene mutation. Affected individuals are at risk of developing various benign and malignant tumors of multiple organs, reviewed in this article. Despite a decrease of number of necropsy, this case as the review of literature demonstrates clinical importance of autopsy.