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Celiac disease is an extremely common, although underdiagnosed, disorder. Knowledge about the varied clinical manifestations and the proper approach to screening and diagnosing celiac disease will lead to appropriate early intervention in affected childrenNew age-dependent algorithms are emerging to properly screen for celiac disease. There is new evidence on the patchy nature of celiac disease supporting the practice of multiple duodenal biopsies including the bulb of the duodenum. Therapeutic dietary compliance, particularly in asymptomatic children, can be poor, and therefore, the involvement of a dietician trained in celiac disease is instrumental in keeping patients up to date with dietary guidelines and to improve their compliance to the diet. Expanding knowledge about the pathogenesis of celiac disease has led to the development of investigational therapeutic alternatives to the gluten-free diet. Ongoing clinical trials are evaluating methods of celiac disease prevention in at-risk infants.This review aims at outlining the different manifestations of celiac disease in children as well as a step-wise approach to screen and diagnose the disease. A better understanding of the pathogenic mechanisms of celiac disease is paving the way to innovative diagnostic tools, preventive strategies, and therapeutic interventions alternative to a gluten-free diet.