This retrospective chart review describes pediatric patients with acute lymphoblastic leukemia or acute myeloid leukemia diagnosed between January 1999 and January 2008, who were identified with enteritis, typhlitis, or colitis. Among the acute leukemia patients, 33/449 (7.3%) with acute lymphoblastic leukemia and 13/89 (14.6%) with acute myeloid leukemia experienced 51 episodes of enteritis (n=8), typhlitis (n=15), colitis (n=19), or enterocolitis (n=9). Twenty-five (49%) patients were exposed to corticosteroids within 14 days of the episode and 35 (68.6%) had fever and neutropenia concurrent with the episode. Forty-eight (94%) patients were treated with complete bowel rest and broad-spectrum antibiotics. However, 3 patients received no therapy and had uneventful courses. Complications included sepsis in 7/51 (13.7%) and intestinal obstruction in 3/51 (5.9%). One child required surgery for abscess drainage and 2 children died of causes unrelated to their colitis. Enteritis, typhlitis, or colitis occurred in 8.6% of children treated for leukemia. The optimal management approach is uncertain.