Carnosine is a vital endogenous dipeptide that has anti‑inflammatory, antiaging, anti‑crosslinking, antitumor and immune regulatory effects. Numerous cell and animal model studies have proved that carnosine and its compounds promote the proliferation and differentiation of osteoblasts, inhibit osteoclasts and protect chondrocytes. They also regulate the cell cycle of bone progenitor cells and the differentiation of bone marrow mesenchymal stem cells, accelerate fracture healing, delay bone tumor development and ameliorate osteopenia induced by estrogen deficiency or disuse. The correlations between carnosine and activation signal molecules, pluripotent differentiation of bone marrow mesenchymal stem cells and interaction between bone cells are unclear. However, studies have proved that carnosine and its compounds have benefits in preventing and treating specific bone diseases. This makes them potential agents for the treatment of osteoporosis and bone tumors. The present review summarized the existing research on carnosine and its compounds in bone cells and tissue. It focused on the physiological function of carnosine and its compounds in the bone and their effect on bone metabolism‑related diseases, thus providing support for developing new strategies for targeted therapy.