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The aim of study was to perform a comprehensive review of the pathogenesis, available diagnostic procedures, prevalence, clinical manifestations, and consequences of small bowel bacterial overgrowth (SBBO) as well as treatment options in the pediatric population.A literature search including MEDLINE, PubMed, and Web of Science databases was performed.SBBO is found in a variety of childhood conditions in which the normal homeostatic mechanisms restricting bacterial colonization in the small bowel are disturbed by congenital or acquired anatomical abnormalities, diminished gastric acid secretion, congenital alteration of intestinal motility or acquired small bowel diseases, or other chronic disorders including primary or acquired immunodeficiency. Data show that SBBO may be an underrecognized cause of pediatric morbidity. Although several diagnostic tests for SBBO determination are available, each has its drawbacks and limitations. Indeed, there is still no “criterion standard” for SBBO diagnosis in the pediatric population. Owing to lack of established guidelines and few published interventional studies that assess the effectiveness of SBBO therapy, treatment of children with SBBO remains empiric and comprises antibiotic or probiotic therapy.Further research is needed to determine the clinical impact of SBBO and to establish diagnostic and therapeutic guidelines applicable to children.