Stiff-person syndrome is a rare neurological disease with little anesthesia experiences. No research shows the definite anesthetic could be used safety in stiff-person syndrome. Post operative hypotonia was described in previous case reports, which was assumed to relate to the interaction of muscle relaxants and inhalation anesthetics with baclofen. This review is summarized the clinical characteristics, main pathological mechanisms and previously anesthesia experiences of stiff-person syndrome.