Abstract: Objective To systematic review the methodological quality of guidelines for Guillain-Barré syndrome (GBS) in children, to provide a reference for clinical evidence-based medicine. Methods Guidelines concerning GBS were electronically retrieved from PubMed, EMbase, CBM, Wanfang data, CNKI and Vip. The guide databases includes major medical institutions and industry sites such as NGC, GIN, TRIP , CDC, IDSA, AAP, WHO, Chinese Health and Family Planning Committee website, library of clinical guidelines China and Chinese clinical guidelines for collaboration. All the data were searched from inception of the database or network to Oct. 2013. Two reviews independently screened literature according to the inclusion and exclusion criteria, and assessed the quality of guideline using the AGREEⅡ . Intraclass correlation coefficient (ICC) was used to examine the conformance of the raters' evaluation scores. Results A total of 5 guidelines concerning GBS were included, with a time range from 2003 to 2012, and origins from USA, EU and Canada. The final recommendation levels of the 5 articles were level B. According to the AGREEⅡ, domain 3 and 4 showed the higher scores, and scores were generally low in domain 5. Plasma exchange (PE) and intravenous immuneglobulin (IVIG) showed positive effects on the treatment of GBS. But it was not recommended that combined PE and IVIG. Corticosteroids are also not recommended for GBS treatment. Conclusions The recommendations of medicines for GBS are basically consistent. However, the classification criteria of the levels of evidence and recommendation are still unconsistent and suboptimal. The guidelines on GBS should be improved in “Applicability” in future.