Patients with cerebral ischemia or hemorrhage due to fibromuscular dysplasia (FMD) should be admitted to a monitored hospital bed and receive supportive stroke care. Based on our personal clinical experience, we recommend antiplatelet agents for future stroke prevention in patients with symptomatic FMD. In patients with watershed stroke due to hemodynamically significant FMD, our opinion is that hypertensive, hypervolemic therapy should be initiated immediately. Additionally, we suggest that potential revascularization therapies, such as intraoperative or primary percutaneous angioplasty, should be discussed.