Abstract: Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy clinically characterized by symmetrical limb weakness and sensory disturbances. Severe cases may develop respiratory muscle paralysis, potentially becoming life-threatening. Campylobacter jejuni (C. jejuni) is a microaerophilic, Gram-negative, curved short rod bacterium. As a common etiologic agent of infectious diarrhea globally, it is also recognized as the most critical pathogen associated with antecedent infections in GBS, posing a substantial threat to human health. The pathogenesis of GBS involves molecular mimicry between C. jejuni lipopolysaccharide epitopes and host gangliosides, which in turn elicits immune-mediated damages to the peripheral nervous system. This review summarizes the molecular mechanisms underlying GBS induced by C. jejuni, as well as recent advances in C. jejuni detection technologies, so as to provide a scientific evidence-based proof for C. jejuni precise control and prevention.