AIM:To review pediatric cases of orofacial granulomatosis(OFG),report disease characteristics,and explore the association between OFG and Crohn’s disease.METHODS:We conducted a systematic review according to the PRISMA guidelines.We searched Medline,LILACS,Virtual Health Library,and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range(<18 years),with no language limitations.All relevant articles were accessed in full text.The manual search included references of retrieved articles.We extracted data on patients’characteristics,disease characteristics,association with other diseases,and treatment.We analyzed the data and reported the results in tables and text.RESULTS:We retrieved 173 reports of OFG in children.Mean age at onset was 11.1±3.8 years(range:2.0-18years).Prevalence in males was significant higher than in females(P<0.001),with a male:female ratio of 2:1.Gastrointestinal signs or symptoms were present in 26.0%of children at the time of OFG diagnosis.Overall,70/173(40.4%)children received a concomitant diagnosis of Crohn’s disease.In about half(51.4%)of the cases the onset of OFG anticipated the diagnosis of Crohn’s disease,with a mean time between the two diagnoses of 13.1±11.6 mo(range:3-36 mo).Overall,21/173(12.1%)of the children with OFG had perianal disease,while 11/173(6.4%)had a family history of Crohn’s disease.Both perianal disease and a family history of Crohn’s disease were significantly associated with a higher risk of Crohn’s disease diagnosis in children with OFG[relative risk(RR)=3.10,95%confidence interval(CI):2.46-3.90;RR=2.74,95%CI:2.24-3.36,P<0.0001 for both).Treatment of OFG included steroids(70.8%of children)and other immunosuppressive drugs(42.7%),such as azathioprine,thalidomide and infliximab.CONCLUSION:High prevalence of Crohn’s disease in children with OFG suggests that OFG may be a subtype of Crohn’s disease. AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease. METHODS: We conducted a systematic review according to the PRISMA guidelines. We searched Medline, LILACS, Virtual Health Library, and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range (<18 years), with no language limitations. All relevant articles were accessed in full text. The manual search included references of retrieved articles. We extracted data on patients We analyzed the data and reported the results in tables and text. RESULTS: We retrieved 173 reports of OFG in children. Men age at onset was 11.1 ± 3.8 years (range: Prevalence in males was significantly higher than females (P <0.001), with a male: female ratio of 2: 1. Gastrointestinal signs or symptoms were present in 26.0% of children at the time of OFG diagnos was. Overall, 70 of 173 (40.4%) children received a concomitant diagnosis of Crohn’s disease. About half (51.4%) of the cases the onset of OFG anticipated the diagnosis of Crohn’s disease, with a mean time between the two diagnoses of 13.1 ± 11.6 mo (range: 3-36 mo). Overall, 21/173 (12.1%) of the children with OFG had perianal disease, while 11/173 (6.4%) had a family history of Crohn’s disease. and a family history of Crohn’s disease were significantly associated with a higher risk of Crohn’s disease diagnosis in children with OFG [relative risk (RR) = 3.10, 95% confidence interval (CI): 2.46-3.90; RR = 2.74,95% CI : 2.24-3.36, P <0.0001 for both. Treatment of OFG included steroids (70.8% of children) and other immunosuppressive drugs (42.7%), such as azathioprine, thalidomide and infliximab.CONCLUSION: High prevalence of Crohn’s disease in children with OFG suggests that OFG may be a subtype of Crohn’s disease.