Clinically, central positional nystagmus (CPN) is often suspected when atypical forms of its peripheral counterpart, i.e., benign paroxysmal positional vertigo (BPPV), are observed, namely a linear horizontal nystagmus as in horizontal canal BPPV or a downwardly and torsionally beating nystagmus as in anterior canal BPPV. Pathophysiologically, CPN is caused by cerebellar and/or brainstem dysfunction. Recent work has provided further insights into the different clinical phenotypes and the underlying pathomechanisms. We performed a PubMed review focused on the findings on CPN using the key words "Central Positional Nystagmus", "Central Positional Vertigo", "Positional Nystagmus" OR "Positioning Nystagmus" OR "Positional Vertigo" OR "Positioning Vertigo" AND "Central" from January 2015 to August 2021. CPN may account for up to 12% of patients with positional nystagmus. Clinical data on CPN are mostly based on case reports or small retrospective case series. CPN is frequently associated with cerebellar and/or brainstem structural lesions, namely stroke, tumours or demyelination, or diffuse involvement of these structures due to degenerative or autoimmune/paraneoplastic diseases; it is also found in patients with vestibular migraine. CPN can be paroxysmal or persistent. The direction of the nystagmus is often downward in head-hanging or apogeotropic in lateral supine positions; combinations of both forms also occur. Clinically it is important to note that CPN is often associated with other central, often cerebellar ocular motor or other neurological signs; typically, it is not improved by the therapeutic liberatory manoeuvres for BPPV. These additional features are also important for the diagnosis, in particular if no structural lesions are found. Pathophysiologically, CPN is believed to reflect an abnormal integration of semicircular canal-related signals by the cerebellar nodulus, uvula and/or tonsil, ultimately providing an erroneous estimation of the head tilt and/or eye position coordinates. The natural course of CPN remains, so far, largely unknown. Symptomatic treatment of CPN consists of pharmacotherapy, e.g., with 4-aminopyridine, and causative treatment of the underlying disease if known. CPN is an important differential diagnosis to BPPV and a clinically relevant entity with heterogenous clinical presentations and various pathomechanisms and etiologies. In particular, studies on the natural course and treatment of CPN are needed.