Diboride-based ultra-high temperature ceramics (UHTCs) are a special class of ceramics with excellent comprehensive properties, which have extensive potential applications in extreme environments. However, their practical applications are limited, mainly due to the poor fracture toughness and thermal shock resistance. Refractory metals have high melting points, good ductility, and high toughness, which have huge potential to improve the properties of diboridebased ceramics. As a special class of additives, they have been adopted to promote densification, improve microstructure, and properties. However, diboride-based ceramics containing refractory metals have not received adequate attention due to relatively weak practical effects on property improvement. The present review highlights the progress and existing problems of transition metal diborides with refractory metal additives, including W, Ta, Mo, Nb, Hf, V, Cr, and Zr, focusing mainly on the microstructure change and property improvements, followed by challenges and possible future development strategies.