Moyamoya disease (MMD), fibromuscular dysplasia (FMD), and cerebral artery dissections represent rare yet clinically significant causes of cerebral ischemia. Timely and accurate diagnosis, followed by appropriate management including surgical and interventional therapies, is essential for better patient outcomes. This chapter reviews the pathogenesis, clinical considerations, and current management paradigms for these rare cerebrovascular conditions, highlighting the need for individualized treatment plans and advancements in surgical and interventional techniques. MMD is a rare cerebrovascular disorder characterized by progressive stenosis of the intracranial arteries around the circle of Willis, accompanied by the development of abnormal collateral vessels, so-called Moyamoya vessels. These vascular changes predispose patients to ischemic strokes and intracranial hemorrhages in pediatric and young adult populations. Currently, extracranial-intracranial revascularization surgery remains the only evidence-based intervention to reduce stroke recurrence. This article discusses the latest indications, surgical techniques, and perioperative management strategies for MMD. FMD is another rare vascular disease affecting systemic arteries, including extracranial cranio-cervical, renal, and iliac arteries. In the context of cranio-cervical involvement, FMD can result in cerebral ischemia, necessitating a combination of pharmacological therapies and, in some cases, surgical or endovascular interventions. Arterial dissections, which can occur in both intracranial and extracranial vessels such as the internal carotid and vertebral arteries, may lead to cerebral infarction or subarachnoid hemorrhage, especially when associated with aneurysmal changes. Of particular concern are blood blister-like aneurysms of the internal carotid artery, which pose significant therapeutic challenges due to their fragility and complexity.