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Celiac disease is a lifelong immune-mediated systemic disorder that may develop in genetically predisposed individuals when exposed to dietary gluten. Its prevalence is estimated to be 1-3% in the European Community; in particular in children studies report a prevalence ranging from 1/500 to 1/93. Awareness of its wide clinical spectrum is mandatory and relevant to all physicians, and in particular pediatricians, to allow a prompt diagnosis and therapy. While the first european guidelines recommended the need of 3 consecutive duodenal biopsies to establish a diagnosis, from 2012 an histological assessment is not considered necessary in certain circumstances in children. Gluten free diet is still the only therapy available in celiac disease disease but dietary adherence allows control of symptoms and reduces the risk of malignancy. However this diet is challenging due to its costs, quality of life implications, and health consequences such as obesity. Therefore alternative therapies are currently being developed. This review will focus on the current knowledge on celiac disease in children.