We report 3 cases of spontaneous cervical epidural hematoma with sudden onset of neck pain followed by the development of unilateral limb weakness. All of the patients were initially suspected to have acute ischemic stroke. We considered using intravenous thrombolysis with recombinant tissue plasminogen activator (rt-PA) to treat 2 of the 3 patients who had arrived at our hospital within 2 hours of the symptom onset. However, we did not administer rt-PA therapy to these patients because the symptoms were mild. We treated all 3 patients with other antithrombotic drugs until the diagnosis of cervical epidural hematoma was confirmed. Patients with spontaneous cervical epidural hematoma usually present with acute neck pain followed by the development of bilateral limb weakness and urine retention; unilateral limb weakness is rare. Patients with this uncommon presentation must be distinguished from stroke.