Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy associated with progressive limb weakness and areflexia. Up to now, a few cases of GBS following infective endocarditis (IE), particularly prosthetic valve endocarditis, have been reported. We present a case of a 63-year-old male patient in whom GBS developed following aortic prosthetic valve endocarditis. Although GBS is not frequently encountered in patients with IE, we believe that every clinician should consider the probable GBS in those patients suffering from distal paresthesis, progressive limb weakness and ataxia.