Abstract: Lipids play biological roles in brain growth and development, including energy storage, cellular signal transduction, hormone synthesis, and homeostasis maintenance. Lipid dysregulation is closely associated with the occurrence and progression of neurodegenerative diseases, making brain lipids important biomarkers for assessing early diagnosis and prognosis of neurological disorders. This article reviewed abnormal lipid metabolism in Alzheimer′s disease, Parkinson′s disease, and multiple system atrophy, focusing on the possible mechanisms of interaction between β-amyloid (Aβ), α-synuclein (α-syn), phosphorylated Tau protein, and associated lipids and their metabolites. It also summarized key targets for improving neurodegeneration through intervention in brain lipid metabolism pathways.