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Lymphocytic esophagitis (LE) is a term recently suggested for the finding of >20 intraepithelial lymphocytes/high-power field in an esophageal biopsy with no more than a rare granulocyte. Two prior studies of LE suggested an association of LE with Crohn's disease (CD) in young patients, but there has been no systematic review of a large pediatric cohort to determine the prevalence and clinical associations of LE in children.All esophageal biopsies performed at a tertiary care pediatric medical center in 2005 were identified (580 biopsies from 545 unique patients). A blinded histologic review was performed to identify LE cases (>50 intraepithelial lymphocytes/high-power field; <1 granulocyte/50 intraepithelial lymphocytes). Clinical characteristics, endoscopic findings, and follow-up data for each case were reviewed independently by a pediatric gastroenterologist.Thirty-one patients with LE (5.7%) and 49 patients with CD (8.9%) were found among the 545 patients. Six of the 31 LE patients (19%) and 43 of the 514 non-LE patients (8.4%) had CD (P < 0.05). The remaining LE patients had various other clinical diagnoses with no significant clinical correlates. LE was identified in 6 of 49 patients with CD (12.2%) and 25 of 496 patients without CD (5.0%) (P < 0.05). Patients with both LE and CD had a more prominent lymphocytic infiltrate than LE patients without CD.LE seems to be more prevalent in children than in adults and has a significant association with CD in this age group.