Carnosine is an important neuromodulator that was discovered a century ago. Its neuroprotection ability is due to its pleiotropic properties that make it an interesting molecule and thus is being studied in various neurological disorders. In this chapter, the various properties of carnosine that imparts it neuroprotection ability are discussed. Furthermore, the mechanisms underlying various important neurological disorders and the mode of action of carnosine in these diseases, with emphasis on its properties, have been discussed in detail. In addition, the proteins that are involved in these mechanisms that are influenced by carnosine either directly or indirectly have been described.