Enteroaggregative Escherichia coli (EAEC) is an emerging pathogen that causes enteric and food-borne infectious diseases. Children throughout the world appear to be susceptible to EAEC infection. EAEC pathogenesis involves the following three stages: 1) adherence to the intestinal mucosa; 2) increased production and deposition of a mucus biofilm; and 3) mucosal toxicity due to inflammation and cytokine release. The HEp-2 cell adherent assay allows identification of EAECs characteristic aggregative or "stacked brick" adherence pattern. Antimicrobial treatment of children who develop an EAEC infection should be individually based. All children with EAEC diarrhea should receive adequate oral fluid hydration. For children who have persistent diarrhea and severe dehydrating illness despite having received adequate oral rehydration, antimicrobials may be initiated. Azithromycin and rifaximin have been shown to shorten the course of EAEC diarrhea in adults and probably represent the recommended antimicrobials of choice for children with severe or persistent illness. The objective of this review is to increase awareness of this important emerging pathogen and to discuss the epidemiology, pathogenesis, and pathogen and host factors associated with EAEC infection in children.