The incidence of ischemic stroke among young people has significantly increased, with a complex pathogenesis. Studies have shown that autoimmune diseases are related to ischemic stroke, involving factors such as atherosclerosis, atrial fibrillation and valvular heart disease, vasculitis, and hypercoagulable states. This review primarily revisits autoimmune diseases associated with ischemic stroke in youth, especially neurological disorders, and explores their potential pathogenic mechanisms.