Gastrointestinal complications in pediatric acute myeloid leukemia (AML) have not been systematically described in the literature. Our objective was to describe complications related to the small and large bowel in children with AML. Literature searches were conducted of Ovid Medline from 1950 to November 2009 and EMBASE from 1980 to November 2009. We included any study design that described gastrointestinal complications in children and/or adults with AML. Common gastrointestinal complications were typhlitis and enterocolitis. Less common complications included appendicitis, pneumatosis intestinalis, and perianal infections. Both leukemia infiltration and intensive chemotherapy likely play a role in the etiology of these conditions. There is a paucity of carefully conducted studies that describe the natural history of typhlitis and enterocolitis and evidence is needed to help guide the management of gastrointestinal complications. Gastrointestinal complications are relatively common in children with AML. Conduction of carefully performed cohort studies is needed to better understand the spectrum of symptoms and expected consequences of gastrointestinal complications. Randomized trials are required to develop evidence-based guidelines for the management of gastrointestinal complications in pediatric AML.