Inflammatory bowel diseases are the most common and most significant chronic disorders in pediatric gastroenterology. They are not just a disorder of one organ system, but a multisystemic diseases. Inflammatory bowel disease together with medications that are used and other medical or surgical interventions can have adverse effects on linear growth, pubertal development and nutritional status. It has been estimated that approximately 25% of all new patients with inflammatory bowel disease are diagnosed before 20 years of age and 35% of them will be presented during their lifetime with at least one extraintestinal manifestations (1,2). Intestinal manifestations are most commonly manifested as abdominal pain, weight loss, diarrhea, a palpable abdominal mass, hematochezia and appearance of perianal fistulae or abscesses. On the other hand, extraintestinal manifestations may involve skin, joints, eyes, respiratory and hepatobiliary systems, as well as increased risk for venous thrombosis. It is important to be aware of exstraintestinal manifestations because it could be the first presentation of the disease even before gastrointestinal symptoms develop. It is important to emphasize that extraintestinal manifestations do not consistently correlate with the degree of intestinal inflammation but if they occur together with the intestinal disease, then they will increase mortality in this children. Inflammatory bowel disease can also contribute to a greater risk for the development of a variety of psychosocial problems. All these mentioned problems can interfere with their ability to participate in their daily life activities. The aim of this review is to inform clinicans about the frequency of exstraintestinal manifestations in the pediatric population and to describe some of the features of inflammatory bowel disease unique to this population. These features could potentially have a significant impact both in the further research as well as clinical management of patients with inflammatory bowel diseases.