A 48-year-old woman with a 29-year history of pulseless disease presented with subarachnoid hemorrhage caused by a rare ruptured intracranial aneurysm of the right vertebral-posterior inferior cerebellar artery. The aneurysm was successfully clipped. Pulseless disease is a chronic and nonspecific vasculitis involving mainly the aorta and its main branches such as the carotid, subclavian, vertebral, and coronary arteries. The cervical vessels are mostly affected in Japanese patients. Almost all patients have ischemic disorders of the involved vessels, manifesting as syncope, visual disturbance, or a faint or absent pulse. The mechanisms responsible for pulseless disease and intracranial aneurysms are likely to be different.