There are few reports describing young-onset amyotrophic lateral sclerosis (ALS). Age at onset is a prognostic factor in ALS, and thus it is relevant to investigate the clinical features of very young ALS patients. We describe three young-onset ALS cases and review the literature. SOD1 mutations were not identified. Our cases and 24 others from the literature indicate that young-onset ALS is characterized by slowly progressive symmetrical weakness; nevertheless, progression is variable. Young-onset ALS seems to be a distinct clinical syndrome but its aetiological background is largely unknown.