Amyotrophic lateral sclerosis (ALS), which has the main syndrome of myasthenia and muscular atrophy, belongs to the category of "flaccidity disease" and "spleen bi" in traditional Chinese medicine. The ALS belongs to the category of "apoplectic aphasia" and "pyretic aphasia with sudamina" with the main syndrome of medullary paralysis. In traditional Chinese medicine, it is believed that the main pathogenesis of ALS is asthenia in origin and sthenia in superficiality, and the five zang viscera can cause flaccidity disease. The most common disease site is spleen and stomach, and the treatment mostly begins with replenishing spleen and stomach. It helps to delay the progression of muscular atrophy and bulbar palsy, and prolong the survival time of patients. In this paper, the historical evolution of ALS is discussed from the point of view of traditional Chinese medicine, and the pathogenesis, diagnosis and treatment of ALS and its research progress are summarized.