Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease, and its pathogenesis is complex and has not been fully elucidated. In recent years, the dysregulation of the gut-brain axis (GBA) is considered to play an important role in the pathogenesis and progression of ALS. Intestinal microbiota and their metabolites, intestinal barrier function, immune system activation, and neuroinflammation communicate bidirectionally with the central nervous system through GBA, thereby influencing the pathological process of ALS. Meanwhile, various active components of Chinese herbal medicine have shown significant potential in regulating GBA function, improving gut microenvironment, and alleviate neuroinflammation. For instance, berberine can activate autophagy to clear TAR DNA-binding protein 43 (TDP-43) aggregates; ginsenoside and its non-saponin component Gintonin can inhibit the TLR4-LPA1 signaling pathway to regulate neuroinflammatory responses; ligustrazine derivative tetramethylpyrazine nitrone (TMPN) can enhance the mitochondrial antioxidant function mediated by PGC1-α (peroxisome proliferator-activated receptor gamma coactivator 1-α); the 7,8-dihydroxyflavone (7,8-DHF) prodrug R13 can activate the TrkB-AMPK pathway to improve neuronal energy metabolism. This paper systematically reviews the mechanism of GBA in the pathogenesis and development of ALS, and focuses on discussing the potential mechanisms and research progress on Chinese herbal medicine interventions for ALS through the modulation of GBA function, aiming to provide new strategies and insights for ALS treatment.