Neutropenic enterocolitis, also known as typhlitis or ileocecal syndrome, is a rare, but important, complication of neutropenia associated with malignancy. It occurs as a result of chemotherapeutic damage to the intestinal mucosa in the context of an absolute neutropenia and can rapidly progress to intestinal perforation, multisystem organ failure, and sepsis. Presenting signs and symptoms may include fever, abdominal pain, nausea, vomiting, and diarrhea. Rapid identification and timely, aggressive medical and/or surgical intervention are the cornerstones of survival for these patients.