Abstract: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive loss of motor neurons in the brain and spinal cord. Currently, causative pathogenic mechanisms in ALS remain poorly understood. Accumulating evidence suggests that neuroinflammation plays an important role in the pathological mechanisms of ALS. This article reviews the role of key cells involved in neuroinflammation during the pathogenesis of ALS, including microglia, astrocyte and peripheral immune cells, and summarizes the clinical development of drugs targeting neuroinflammation of ALS, aiming to provide new directions for the pathological research on ALS and the research and development of related drugs.