Fabry's disease (FD) or angiokeratoma corporis diffusum, is a rare X-linked inherited disorder of glycosphingolipid metabolism. This chapter analyzes the clinical, radiologic, and pathologic features of hemizygote and heterozygote FD patients and cerebrovascular involvement based on a comprehensive review of literature. Neuropathologic autopsy findings are consistent with prior events of cerebral ischemia and, rarely, hemorrhage. In FD, in addition to cerebral ischemia, dolichoectatic intracranial arteries may also cause neurovascular compression syndromes. Triventricular hydrocephalus related to dolichoectatic basilar artery has been reported in hemizygotic and heterozygotic patients. Magnetic resonance imaging (MRI) studies show progression of white-matter lesion load despite enzyme replacement therapy (ERT), although these patients did not show clinical neurologic progression. Administration of antiplatelet agents may help to prevent the atherosclerotic and thromboembolic effects of damage to the vascular endothelium, but experience with this approach is limited and the use of oral anticoagulant agents should also be considered.