Neutropenic Enterocolitis ( Typhlitis) is a rare, life-threatening gastrointestinal complication seen in patients with hematologic malignancies. Severe neutropenia, infections or chemotherapy alter the bowel mucosal surface which can proceed to necrosis and perforation. We report 4 cases of pediatric patients diagnosed with acute lymphoblastic leukemia treated with myelosuppressive chemotherapy, who developed severe neutropenic enterocolitis. According to BFM ALL 2002 risk stratification, 3 patients were included in High Risk group and one in Standard Risk group. All children had received chemotherapy before the diagnosis of typhlitis; at the time of the diagnosis, 2 patients had bone marrow relapse, while the other 2 had chemotherapy induced bone marrow aplasia. 3 patients presented the typical triad of neutropenia, fever and abdominal pain. Most frequent clinical features included abdominal pain, diarrhea and abdominal distention. Abdominal ultrasonography scan identified diagnostic features consistent with typhlitis. One patient presented surgical condition such as bowel perforation. 3 patients died despite prompt diagnosis and treatment with broad spectrum antibiotics, antifungal agents, granulocyte colony-stimulating factor, bowel rest, parenteral nutrition, and platelet units concentrate and packed red cell transfusions. The diagnosis of typhlitis was determined by clinical features and was supported by radiologic evaluation. The course can be towards the sepsis syndrome, multisystem organ failure and death. Oncologic patients presenting with fever, abdominal pain and neutropenia are more likely to develop typhlitis.