Central positional nystagmus (CPN) is caused by disorders affecting the central vestibulo-cerebellar pathways. When associated with prominent vertigo, oscillopsia, and/or dysautonomia, treatment directed towards the underlying disorder, the underlying mechanism, and/or short-duration vestibular suppressants and antiemetics might be offered, albeit with heterogeneous results. There is a suggestion that drugs hypothetically targeting the CPN mechanism, including clonazepam, baclofen, and aminopyridines, might improve CPN in the short term. However, long-term evaluation of CPN natural progression and its response to treatment is largely unknown. Moreover, treatment response of associated subjective positional vertigo and/or related symptoms is also lacking, as well as a standardized assessment for its evaluation. In summary, prospective randomized placebo-controlled treatment trials using objective and standardized baseline, short- and long-term assessment of CPN and related symptoms are highly needed.