Introduction: Although indeterminate colitis (IC) has a reported prevalence of 10% of adult patients with Inflammatory bowel disease (IBD), the true prevalence in children is unknown. Our aim is to determine the prevalence of IC in our pediatric population of patients with IBD and to describe its clinical presentation and disease distribution Methods: We performed a retrospective database analysis of all children diagnosed with IBD at The Johns Hopkins Children’s IBD Center between 1996 and 2003. All available patient demographics, including age, sex and age at disease onset was tallied. Disease distribution was identified based on a review of all endoscopic, colonoscopic, radiological and histopathological records of all patients contained within the patient database. Results: In total, 250 pediatric patients (3:4;M: F) are contained within the patient IBD database. 75 (29%) were initially diagnosed with IC based on histopathology at the time of diagnosis. The age at disease onset was 7.1yrs and an average of a 4-month lag to diagnosis from the time of symptoms onset. Family history of IBD was present in 17.1%. These patients presented clinically with a number of clinical symptoms, including abdominal pain (52%), bloody stools (44%), diarrhea (28.3%) and poor growth (17.5%). Among these patients, 50 (67.5%) maintained the diagnosis of IC. The remaining 25 patients obtained the diagnosis of either Crohns disease (CD) (16) or Ulcerative Colitis ( UC) (9) within 1.2 years from the time of diagnosis. There was no predictive clinical, biochemical, endoscopic or histological criteria that differentiated these various sub-groups. Sixty-one patients have total colon involvement. 13 (17.5%) patients showed a disease progression from the time of diagnosis from either left sided, or recto sigmoiditis to total colon involvement. Conclusion: Indeterminate colitis is a significant subcategory of pediatric patients with IBD with a prevalence that is much higher than observed in adults. These patients also show an earlier age at diagnosis than patients with Crohn’s disease. Most patients present with pancolitis at the time of diagnosis or progress rapidly to total colonic involvement. Longitudinal studies are needed to determine the natural progression and surgical implications of this important subtype of pediatric IBD.