Amyotrophic lateral sclerosis (ALS) is a life-threatening neurodegenerative disease causing progressive degeneration of motor neurons, ultimately resulting in death. Till now, no medicinal strategy has been proved to be completely successful in ameliorating the disease’s symptoms, except riluzole that only has a moderate effect. A limited therapeutic intervention of ALS encourages patients to opt for alternative medicine and therapies. Traditional Chinese herbal medicines (TCM) have been shown to overcome neuroinflammation, excitatory amino acid toxicity, oxidative stress, apoptosis and autophagy. In this regard, Chinese herbal medicines (CHMs) have been explored as a therapeutic option to manage clinical manifestations of ALS and other neurological diseases. In this review, we summarize the therapeutic benefits of CHMs on various neurodegenerative disorders, particularly ALS. The mechanistic details of various Chinese herbs along with their active molecules that delay the disease onset have also been discussed.