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Background Jaundice is a life-threatening disorder in the neonates.In the present study,we aimed to assess systematically available evidence on causes and management of jaundice in Iranian newborn patients.Methods We searched the databases of PubMed,Web of Sciences,Scopus and Google Scholar for English articles published since inception until May 2019.A search was also done for Persian articles in Magiran and Scientific Information Database.Studies were evaluated based on predefined criteria by two reviewers.Data analysis was performed by STATA software.Results A total of 33 articles were finally included.The overall pooled prevalence of causes of jaundice among Iranian neonates was as follows: ABO blood groups incompatibility,16.9％[95％ confidence interval (CI) 10.9-22.8];Rh blood group incompatibility,4％ (95％ CI 2.5-5.5);ABO and Rh blood groups incompatibility,3.6％ (95％ CI 0-7.7);glucose6-phosphate dehydrogenase (G6PD) deficiency,6.3％ (95％ CI 5.1-7.5);infection,6.6％ (95％ CI 5.2-8.1);hypothyroidism,4.2％ (95％ CI 0.1-8.3);infant of diabetic mother: 2.3％ (95％ CI 0.1-4.5);unknown,50.7％ (95％ CI 33.4-68);cephalohematoma,0.6％ (95％ CI 0.3-0.9).Regarding treatment of icterus,seven and eight articles were found on phototherapy and exchange transfusion,respectively.In five studies,all patients underwent phototherapy,but rate of exchange transfusion use was between 6.6％ and 50.9％.Conclusions According to the results,unknown factors were the most common causes of icterus in Iranian neonates,followed by ABO blood groups incompatibility,infections and G6PD deficiency.By the way,phototherapy and exchange transfusion were found as therapeutic choices of neonatal jaundice.