Background: Neutropenic colitis (NC) or typhlitis has an incidence of approximately 5% in patients receiving chemotherapy for malignancy. The precise cause is unknown, but almost all patients are neutropenic; most profoundly so. We reviewed our experience with neutropenic colitis over the past 10 y to assess the incidence, management, and outcome. Materials and Methods: Hospital records were reviewed after obtaining IRB approval (# 10-10-184E). There were 1224 children treated for cancer at our institution over the study interval. Neutropenic colitis was strictly defined as the presence of both clinical and radiographic findings consistent with the diagnosis, since there are no definitive diagnostic criteria. Patients with confirmed appendicitis were excluded. Medical management consisted of bowel rest and/or decompression, broad-spectrum IV antibiotics and anti-fungal coverage, and serial clinical exams and radiographic studies. Clinical deterioration and free air were the primary indications for surgery. Demographic factors, signs, symptoms, clinical presentation, underlying disease process, white blood cell count (WBC), and absolute neutrophil count (ANC) prior to the onset of the disease, treatment, and outcome were analyzed. Results: Neutropenic colitis was confirmed in 17 children (1.4% incidence). Three patients had more than one episode. Leukemia was the most common underlying diagnosis (53%). There was no gender predominance, and the mean age at diagnosis was 8.4 y. Mean WBC and ANC at onset were 670 and 164, respectively. A sharp decline in the ANC usually immediately preceded the onset of NC; 2/17 (12%) underwent operation, but both patients had only pneumatosis at exploration. Overall survival was 9/17 (53%), but no deaths were directly attributable to the colitis. Conclusion: Neutropenic colitis is an uncommon occurrence in children with neoplasia (1.4% in the current study). Leukemia is the most common cause. A precipitous decline in ANC usually occurs prior to the onset of NC. Most patients do not require operation and the overall mortality directly attributable to NC is low. © 2011 Elsevier Inc. All rights reserved.