Abstract: ATP-binding cassette (ABC) transporters are ubiquitous membrane-bound proteins that are responsible for the translocation of a broad spectrum of substrates across cellular membranes, including lipids, amino acids, nucleosides, sugars, and xenobiotics. Interestingly, ABC transporters are highly expressed in the brain. While their functions in the brain still need to be elucidated, several members are implicated in the pathogenesis of neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease (PD), and frontotemporal dementia. In this perspective, we will review current knowledge of ABC transporters in the central nervous system in terms of physiological functions and pathology in neurodegeneration. Furthermore, we will explore the possibilities of ABC transporters as potential targets in the development of therapeutics for neurodegenerative diseases.