Crohn’s disease (CD), with ulcerative colitis (UC) and inflammatory bowel disease unclassified (IBD-U), belongs to the group of inflammatory bowel diseases (IBD), that are chronic, idiopathic, and detrimental disorders of the gastrointestinal tract. CD manifests during childhood or adolescence in up to 25 % of patients, and the incidence has been increasing over the past four decades. Although the etiology and the mechanisms of CD are still unknown, recent data suggest that it is the result of an altered immune homeostasis within the intestinal mucosa in genetically predisposed individuals: This may evolve in an uncontrolled inflammatory response probably triggered by the intestinal microbiota and/or other environmental factors. Presenting symptoms and therapeutic armamentarium are similar in adults and children; however, there are significant peculiarities characterizing pediatric-onset CD. Children with CD are at particular risk for extraintestinal features, including growth failure, delayed puberty, weight loss, and anemia, that may be more harmful than in adults. Therapy itself can adversely impact normal growth and development. Other issues unique to pediatric patients are the paucity of controlled clinical trials and the psychological issues that occur in children and adolescents with IBD.