The annual incidence of neurodegenerative disease has been increasing with the aging of the global population, seriously affecting the quality of life of elderly patients and imposing a heavy burden on society. Glycyrrhetinic acid, which inhibits neuroinflammation and protects neurons, is one of the main active ingredients of the traditional Chinese medicine Glycyrrhiza glabra. Increasing numbers of studies are focusing on the mechanism of action of glycyrrhetinic acid and its derivatives in neurodegenerative disease. This review summarizes studies on the effects and mechanisms of action of glycyrrhetinic acid and its derivatives in Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis, multiple sclerosis, and cerebellar atrophy. Additionally, the future applications of glycyrrhetinic acid and its derivatives in neurodegenerative disorders are discussed.