Celiac disease is an autoimmune enteropathy that occurs in genetically predisposed individuals. Screening is with serum celiac disease-specific antibodies, most commonly anti-tissue transglutaminase IgA (when IgA sufficiency has been assured with normal total serum IgA). Diagnosis is via upper endoscopy with biopsies of the small intestine that show intraepithelial lymphocytosis, villous blunting, and crypt hyperplasia. Treatment is a strict lifelong gluten-free (GF) diet—avoidance of wheat, barley, and rye. Adoption of a gluten-free diet can be difficult for a variety of reasons and education and counseling are best performed with the help of a registered dietitian. Continued monitoring of patients for nutritional deficiencies and ongoing symptoms and growth in children is paramount in management of pediatric celiac disease.