Concerns have been raised about the significant biofouling and environmental problems caused by the large numbers of Limnoperna fortunei clinging to water intake facilities. This review first provides a summary of the occurrence of L. fortunei in typical regions including China, South America, and Japan. Furthermore, this article provides a comprehensive overview of the biological traits, risks, and control of L. fortunei. Importantly, the planktonic larval stage is a critical period for the expansion of L. fortunei. Its biofouling process mainly relies on the adhesion of byssus to substrates. Various physical and chemical methods have been proposed and used to control L. fortunei. Among these methods, sodium hypochlorite has been shown to be effective in preventing the adhesion of L. fortunei by dissolving its byssus at much lower concentrations. Overall, effective and environmental-friendly antifouling strategies are still rare, particularly in drinking water treatment systems, and are encouraged to develop in future studies. This review not only provides a comprehensive understanding of L. fortunei but also helps to guide the prevention and control of L. fortunei.