Abstract. Background. Physical activity and occupational exposures appeared to play a relevant rolein pathogenesis of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease of unknownorigin. Materials and methods. We aimed to make an overview of the clinical characteristics and lifestyle(occupation and sport) of a population of 395 patients with ALS from Campania, in southernItaly. Results. ALS onset resulted anticipated of about 11 years in industry workers, whilst the morefrequent site of onset among farmers was upper limbs. Compared to non-athletes, athletes, particularlysoccer players, showed a 7 years anticipation of ALS onset, with higher mortality after 5 years.Discussion and conclusions. We suggest that subjects genetically prone to abnormal response to hypoxiaduring strenuous physical activity or exposed to neurotoxic agents, such as athletes, farmers orindustry workers, might present increased risk to develop ALS. Future case-control and follow-upstudies on our population should be implemented to deepen the present results.