Infantile lipoma (or lipoblastoma) of the mesentery is an extremely rare benign tumor of embryonal fat, with 15 cases reported in the English literature until today and only three of them arise from the ileum mesentery. We report an 18-month old boy presenting with a palpable intraabdominal mass arising from the ileum mesentery. Histopathologic and cytogenetic studies confirmed the diagnosis of mesenteric lipoblastoma (or infantile lipoma). Complete excision of the mass was performed. A follow-up examination consisting of physical examination and an abdominal ultrasound at 30 months postoperatively revealed no recurrence. We also present a review of the English literature regarding the presentation and management of mesenteric lipoblastomas in children.