Neurodegenerative diseases, of which tauopathies form a major class, are increasing in prevalence worldwide. Tauopathies are characterized by the presence of abnormal neuronal and glial inclusions that are composed predominantly of the microtubule associated protein, tau. Several foci of increased incidence of tauopathies have been identified that have been attributed to environmental neurotoxins. Exposure to inorganic or organic toxins (such as β-Methylamino-L-Alanine and annonacin) through water, food and traditional medical practices is the suspected etiology but it remains to be proven whether these are sole sufficient factors. These progressive and uniformly fatal diseases are characterized clinically by various combinations of dementia, parkinsonism and motor neuron disease. This review attempts to summarize the currently available information on small geographic clusters of endemic environmental tauopathies that have been identified in the Chamarros of Guam, inhabitants of the Kii Peninsula in Japan, members of the Auyu and Jaqai groups in Papa New Guinea, and residents of Guadeloupe. Possible unifying neurodegenerative mechanisms are explored in relation to sporadic tauopathies worldwide.