Pigment dispersion syndrome (PDS) and pigmentary glaucoma (PG) are two stages within the same ophthalmic disease spectrum, which are known to be affected by race. The prevalence of PDS is underestimated, largely due to its minor clinical symptoms. Although the prevalence of PG is low, the visual impairment associated with PG is extremely severe. The prevalence of PDS-PG is four or more times higher in Caucasians than in Blacks or Asians, and the "classic" PDS in Caucasians has long been used as a benchmark diagnostic criterion. Following extensive research focused on African Americans and Asians, the standard for diagnosing PDS-PG was refined. At the same time, the pathogenesis of PDS is not the same in different races. Hence, the effectiveness of preventive treatment and the need for treatment may not be equivalent in different races. The rate of conversion of PDS to PG is nearly 1/3 in Caucasians and higher in blacks and Asians, requiring more aggressive treatment and monitoring. We systematically searched a PubMed database from inception to March 2022 to provide an overview of research progress in various aspects of PDS-PG. Specifically, this paper considers the effects of race on disease prevalence, clinical manifestation, diagnostic criteria, disease mechanism, hereditary traits, treatment, and prevention to provide an accurate and comprehensive guide for the diagnosis and treatment of PDS-PG in various races.