The term "microbiota" dates back to the beginning of the 20th century and refers to a large number of microorganisms, including bacteria, yeasts and viruses, which coexist in various parts of the human body (intestine, skin, lung, oral cavity). Growing evidence has confirmed that the microbiota is associated with the development of numerous diseases, including brain disorders and neurodegenerative diseases. A neurodegenerative disease that has been gaining prominence in the study of microbiota is Amyotrophic Lateral Sclerosis (ALS), which mainly affects the motor system. In this perspective, the present work consists of a narrative, qualitative, exploratory descriptive review with the objective of compiling knowledge about the relevance of the microbiota in the treatment of amyotrophic lateral sclerosis. This is a bibliographic search carried out in February 2023 in the PubMed and Virtual Health Library databases with the descriptors "microbiota", "amyotrophic lateral sclerosis" and "treatment", combined using the Boolean operator AND in the range of time over the past 5 years. This research evidenced the relationship between microbiota and amyotrophic lateral sclerosis in four axes: relation between microbiota and central nervous system, the role of antibiotic use in ALS, microbiota composition in patients with ALS and fecal microbiota transplantation. The findings indicate that the microbiota may play a critical role in the development of amyotrophic lateral sclerosis, given that the influence of the microbiome in the protection of homeostatic microglia with the inhibition of the neurodegenerative phenotype, the presence of certain microorganisms influencing the progression of the disease and consequent degeneration, the role of antibiotic use in the progression of ALS, fecal microbiota transplantation as a therapeutic alternative for the treatment of this pathology and possible therapeutic strategies for ALS. However, research on the relationship between gut microbiota and ALS is still in its early stages, but the fact that the microbiota has been closely associated with other neurodegenerative diseases raises hopes for the future.