Coeliac disease is an immune-mediated enteropathy caused by a permanent sensitivity to gluten in genetically susceptible individuals. It occurs in children and adolescents with gastrointestinal symptoms,dermatitis herpetiformis, dental enamel defects, osteoporosis, short stature, delayed puberty and persistent iron deficiency anaemia and in asymptomatic individuals with type 1 diabetes, Down syndrome, Turner syndrome, Williams syndrome, selective immunoglobulin (Ig)A deficiency and first degree relatives of individuals with coeliac disease. The Coeliac Disease Guideline Committee of the North American Society for Paediatric Gastroenterology, Hepatology and Nutrition has formulated a clinical practice guideline for the diagnosis and treatment of paediatric coeliac disease based on an integration of a systematic review of the medical literature combined with expert opinion.The Committee examined the indications for testing, the value of serological tests, human leukocyte antigen (HLA) typing and histopathology and the treatment and monitoring of children with coeliac disease. It is ecommended that children and adolescents with symptoms of coeliac disease or an increased risk for coeliac disease have a blood test for antibody to tissue transglutaminase (TTG), that those with an elevated TTG be referred to a paediatric gastroenterologist for an intestinal biopsy and that those with the characteristics of coeliac disease on intestinal histopathology be treated with a strict gluten-free diet (GFD).