In recent years, high-entropy alloys (HEAs) have become the research hotspot in the field of metal structural materials because of their novel design concepts and excellent physicochemical properties. With the continuous popularization of lightweight alloy design concepts, the conception of “entropy regulation” has been widely used to develop new lightweight alloys. Lightweight HEAs (LHEAs) are a new type of low density HEAs based on lightweight alloy designs. Their development and design mainly combine the empirical parameter criteria, phase diagram calculations, and first-principles calculations. Al-Ti-V-based LHEAs attract much attention among various LHEAs due to their excellent mechanical properties, good high temperature oxidation resistance, and fine corrosion resistance. This paper summarized the research progress of Al-Ti-V-based LHEAs from the perspective of composition design, preparation methods, microstructures, and physicochemical properties. Meanwhile, the problems and challenges for Al-Ti-V-based LHEAs were also prospected.