Recent statistics report that 3 million people, or 1% of the population in the United States (U.S.), are affected by celiac disease (CD). In addition, in the U.S., as many as 1 in 80 children is affected with CD. However, CD can be challenging to diagnose and many children are not correctly diagnosed or live without a diagnosis for several years. Symptoms, if present, are often nonspecific and may be common manifestations of many pediatric illnesses. The purpose of this review is to examine the current evidence regarding incidence, pathophysiology, diagnosis, and treatment of a child with CD. Clinical implications for nurses caring for children and families are discussed.