Objective To analyze the clinical characteristics of intracranial inflammatory demyelinating diseases manifested as isolated vertigo in 2 cases for improving the disease awareness and levels of diagnosis and treatment of central and peripheral isolated vertigo. Methods The history, physical examination, auxiliary examinations and treatment of 2 patients with isolated vertigo were analyzed and summarized. Results Both patients suffered from acute isolated vertigo. One patient had previous history of upper respiratory infection and another case showed non-fatigue positional nystagmus. Both the two patients showed normal results of neurological system, hearing and vestibular functions, but abnormal signals of brainstem or cerebellum in magnetic resonance imaging (MRI). Steroid pulse therapy was adopted in both patients with good prognosis. Conclusion Diseases of central nervous system should be excluded in acute isolated vertigo as soon as possible.