Positional and positioning vertigo and nystagmus syndromes are usually caused by benign paroxysmal positional vertigo (BPPV). However, it has been recognized sinister central disorders can also lead to positional vertigo and nystagmus in rare occasions. Central positional vertigo (CPV) and nystagmus (CPN) can be caused by cerebellar dorsal vermis lesion, tumors or hemorrhage on the dorsolateral region of the fourth ventricle, degenerative disorders involving cerebellum, and sometimes in migraine. Therefore, the differentiation of benign positional vertigo from sinister central one is important in clinical practice. In the presence of accompanying signs and symptoms suggesting cerebellar or brainstem involvement, the differentiation is straightforward. However, some cases of CPV show only positional vertigo syndrome without accompanying signs or with subtle abnormalities. Efforts have been made to distinguish central from peripheral positional vertigo by clinical features, but only the direction of nystagmus during an attack could allow differentiation. The mechanism of CPN from case-series and animal studies has been postulated the interruption of cerebellar modulation of gravitational pathways by the lesions in dorsal vermis or near the vestibular nucleus. This paper reviewed the clinical characteristics of CPV mainly focused on the differentiation from BPPV.