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In the present review we have discussed the occurrence of β-N-methylamino-L-alanine (BMAA) and its natural isomers, and the organisms and sample types in which the toxin(s) have been detected. Further, the review discusses general pathogenic mechanisms of neurodegenerative diseases, and how modes of action of BMAA fit in those mechanisms. The biogeography of BMAA occurrence presented here contributes to the planning of epidemiological research based on the geographical distribution of BMAA and human exposure. Analysis of BMAA mechanisms in relation to pathogenic processes of neurodegeneration is used to critically assess the potential significance of the amino acid as well as to identify gaps in our understanding. Taken together, these two approaches provide the basis for the discussion on the potential role of BMAA as a secondary factor in neurodegenerative diseases, the rationale for further research and possible directions the research can take, which are outlined in the conclusions.