Progressive muscle paralysis is a hallmark of the heterogeneous neurodegenerative disease known as amyotrophic lateral sclerosis (ALS). The main etiological factors of the disease are complicated interaction of excitotoxicity, mitochondrial failure, oxidative stress, protein aggregation, and neuroinflammation, which results in motor neuron loss and paralysis. ALS is linked to altered gut microbiota, which impacts immunological, metabolic, and neurological processes. The gut microbiome could be crucial in the development of ALS and offers a potential target for therapy aimed at altering excitotoxicity, mitochondrial failure, oxidative stress, protein aggregation, and neuroinflammation, and reducing the speed of disease progression. Recent discoveries in ALS mouse models and clinical studies demonstrate that intestinal dysfunction manifests before the onset of the disease, indicating that a breakdown in gut-neuromuscular communication might play a role in the progression of ALS. Nutritional strategies involving probiotics, prebiotics, or postbiotics could represent promising treatment options for ALS.