The purpose of this report is to discuss the clinicopathologic features, etiologic associations, pathogenesis, and differential diagnosis of lymphocytic gastritis, as illustrated by the case of a 2-year-old female with lymphocytic gastritis and celiac disease. Lymphocytic gastritis is defined by the presence of more than 25 intraepithelial lymphocytes per 100 epithelial cells and is often more prominent in the gastric body compared with the antrum. It is a relatively uncommon disorder, occurring in <5% of gastric mucosal biopsies from adult and pediatric patients with gastritis. Although originally described as the histologic counterpart to “varioliform” gastritis, cumulative data obtained from several clinical and histologic studies indicate that lymphocytic gastritis is not a distinct clinicopathologic entity. Rather, it represents a histologic pattern of injury that may result from various etiologies, most notably celiac disease and Helicobacter pylori infection. The pathogenesis of lymphocytic gastritis remains poorly understood, but emerging evidence indicates that it likely results from an abnormal immune-mediated response to environmental stimuli that develops in genetically predisposed individuals. This form of gastric injury is important to recognize, because pathologists should alert clinicians of the need to exclude the possibility of celiac disease in patients with this disorder.