There have been sporadic reports of aquatic, benthic Microcoleus proliferations in freshwater rivers, lakes, and reservoirs for four decades, with reports increasing in frequency over the last twenty years, suggesting a possible rise in their global distribution, frequency, and intensity. Microcoleus can produce anatoxins which are neurotoxic, and ingestion of toxic mats has caused hundreds of dog fatalities and raised serious human and ecological health concerns. This review synthesizes and evaluates current knowledge on Microcoleus distribution, taxonomy, toxin production, toxicity, ecology, environmental drivers, and biotic interactions. Toxin-producing Microcoleus have been reported in at least 18 countries, though many regions have not conducted toxin testing, suggesting a broader but under-reported distribution. Proliferations occur across diverse habitats, including cobble-bedded streams, large sandy rivers, reservoirs, and lakes. Microcoleus proliferations also occur on macrophytes, both in lakes and rivers. Genomic analyses currently classify anatoxin-producing Microcoleus into distinct species, with all known anatoxin-producers isolated from freshwater ecosystems. Anatoxin concentrations vary widely over space and time, within and among waterbodies. While studies on environmental drivers remain limited, research in cobble-bedded rivers suggests that moderate enrichment of dissolved inorganic nitrogen and low dissolved reactive phosphorus concentrations in the water column promote proliferation. Metagenomic approaches have revealed unique nutrient acquisition and storage strategies used by Microcoleus. Key knowledge gaps remain around the environmental and ecological triggers of proliferation, toxin production, genomic diversity and microbial interactions. Addressing these gaps through coordinated, global studies using robust datasets and consistent methods is critical to improve prediction, monitoring, and mitigation of this increasingly widespread public and ecological health threat.