Polygonisation is a common nonuniform wear phenomenon occurring in railway vehicle wheels and has a severe impact on the vehicle–track system, ride comfort, and lineside residents. This paper first summarizes periodic defects of the wheels, including wheel polygonisation and wheel corrugation, occurring in railways worldwide. Thereafter, the effects of wheel polygonisation on the wheel–rail interaction, noise and vibration, and fatigue failure of the vehicle and track components are reviewed. Based on the different causes, the formation mechanisms of periodic wheel defects are classified into three categories: (1) initial defects of wheels, (2) natural vibration of the vehicle–track system, and (3) thermoelastic instability. In addition, the simulation methods of wheel polygonisation evolution and countermeasures to mitigate wheel polygonisation are presented. Emphasis is given to the characteristics, effects, causes, and solutions of wheel polygonisation in metro vehicles, locomotives, and high-speed trains in China. Finally, the guidance is provided on further understanding the formation mechanisms, monitoring technology, and maintenance criterion of wheel polygonisation.