Background Intestinal lipomas are benign, non-epithelial, intestinal tumors with an exceptionally rare localization at the ileum. Lipomas in the small intestine occur mainly in elderly patients and seldom occur in childhood. They are frequently asymptomatic, possibly due to their slow growth. These tumors may act as a lead point of intussusception. Case presentation We report a rare case of double compounded ileo-ileal intussusception due to a submucosal intestinal lipoma in an 8-year-old female. To our knowledge, this is only the seventh pediatric case to be reported in the medical literature. Conclusions Small intestinal submucosal lipoma should be considered in case of intussusception in pediatric patients. Surgical resection seems sufficient in case of symptomatic intestinal lipoma with low morbidity.