Burkitt lymphoma has many forms of clinical presentations and, in children, it is usually discovered due to the presence of an abdominal mass. This rapidly growing tumor is highly malignant, aggressive, and may cause either indirect symptoms, due to pressure phenomena or direct involvement of the bowel lumen, leading to either intestinal obstruction or intussusception.

We describe the case of a 4-year-old girl who exhibited an unusual presentation of ileocolic intussusception on a Burkitt lymphoma lesion of the ileum.