A case of cerebellar hemangioblastoma with von Hippel-Lindau disease and an aneurysm of the cavernous sinus segment of the internal carotid artery is presented here. A 60-year-old woman presented with a cerebellar solid tumor manifesting as headache of 4 months, progressive vomiting, and ataxia of half a month. Four-vessel angiography revealed a high stain mass tumor located in the posterior fossa; an aneurysm on the cavernous sinus segment of the internal carotid artery was demonstrated at the same time. The ipsilateral anterior inferior cerebellar artery (AICA) and posterior inferior cerebellar artery (PICA) branches were the main feeding vessels of the hemangioblastoma. The patient underwent left-lateral suboccipital craniectomy to remove the highly vascular hemangioblastoma. Successful total excision of the tumor was demonstrated on postoperative magnetic resonance (MR) images. The unruptured cavernous sinus aneurysm in this case was not on the feeding artery, which was asymptomatic and has not been treated further. The patient was in good condition in 1-year follow-up.