Amyotrophic lateral sclerosis is a neurodegenerative disease of unknown cause, which presents with weakness in the limbs or difficulties in speaking or swallowing, which may be associated with a high nutritional risk. Currently there is no cure, so it is approached in a multidisciplinary manner with both pharmacological and non-pharmacological interventions, including nutritional interventions. The aim of the present study was to elucidate the relationship between diet composition and the risk of developing ALS and the evolution of ALS once established. Nutrients and foods that favored the development of ALS (red meat), decreased the risk (antioxidants) and had no effect (mercury), foods and nutrients that favored the progression of the disease (intake deficit) and reduced it (antioxidants) were observed. In conclusion, diet composition and intake of certain micronutrients and foods seem to influence the initial development and progression of ALS.