Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease whichis pathogenically based on the mitochondrial alteration of motor neurons, causing progressive neuron death. While ALS is characterized by enormous oxidative stress, the Mediterranean diet has been seen to have high antioxidant power. Therefore, the aim of this study is to determine how the Mediterranean diet can improve mitochondrial activity, establishing the specific nutrients and, in addition, observing the pathogenic mechanisms related to the disease that would achieve this improvement.To this end, a comprehensive review of the literature was performed using PubMed.KBs have been observed to have a neuroprotective effect to improve energy balance, increasing survival and the number of motor neurons. This ketogenesis can be achieved after following a Mediterranean diet which is associated with great benefits in other neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and ALS. These benefits are due to the high antioxidant power especially based on polyphenols contained mainly in olive oil, wine, nuts, or berries. In short, KBs could be considered as a promising option to treat ALS, representing an alternative source to glucose in motor neurons by providing neuroprotection. In addition, treatment results can be improved as ketogenesis can be achieved (increase in KBs) by following a Mediterranean diet, thanks to the high antioxidant properties which, at the same time, would improve the high oxidative stress that characterizes the disease. K E Y W O R D Samyotrophic lateral sclerosis, ketone bodies, mediterranean diet, mitochondria | INTRODUC TI ONNeurons are cells in charge of transmitting information through chemical and electrical signals, therefore requiring a high level of energy in the brain. This use of energy represents between 20% and 25% of the total oxygen consumed by the body, of which approximately half is used to generate membrane potentials. At the same time, they maintain ion gradients for neurotransmission, the main source of said percentage