Carnosine, a compound with plethora of benefits, was originally discovered in 1900 and is formed by the amide linkage of β-alanine and L-histidine. Carnosine production is limited by β-alanine whereas the imidazole ring of histidine moiety makes it a suitable buffer in physiological pH range. It is reported to be found in the skeletal muscle, brain, heart, and gastrointestinal tissues of humans. This review focuses on the biological properties of carnosine including pH buffering ability, antioxidant activity, anti-inflammatory activity, anti-aging effect, enhancement of cognitive function, and immunomodulation. The relevance of carnosine in muscle function attributing to enhancement of physical performance has also been highlighted. Studies spanning several years have proved the preclinical effectiveness of carnosine in treating diverse pathological diseases. A complete summary of all key activities of carnosine from in vivo investigations and clinical trials has been compiled. Considering its numerous advantages, carnosine may be a promising option for the development of a nutraceutical.