A 53-year-old woman presented with intracranial hemorrhage caused by a ruptured cerebral aneurysm. Digital subtraction angiography revealed a large aneurysm arising from the top of the basilar artery and a Spetzler-Martin grade 3 arteriovenous malformation. Endovascular embolization of the aneurysm was completed with a slight neck remnant. Medical examination confirmed that she suffered from hereditary hemorrhagic telangiectasia (HHT). HHT is a rare autosomal dominant disorder characterized by multiple mucocutaneous telangiectasia and associated vascular malformations. This case suggests that the prognosis for HHT patients with treatable aneurysms should be as good as that of healthy individuals. Consequently, neurosurgeons should suspect underlying HHT in all patients with cerebral vascular malformations.