Abstract. Inflammatory myofibroblastic tumors (IMTs) are rare neoplastic lesions with a tendency for locally aggressive behavior and recurrence. IMTs most frequently occur in the soft tissues of children and young adults, with the lungs being the most commonly affected site; however, it has been recognized that any anatomical location may be involved. IMT in the jejunum is extremely rare, with only one case previously reported in the literature. The current study describes the case of a 42-year-old woman presenting with intermittent abdominal pain and small bowel intussusception that was identified during a laparotomy. Surgical resection of the jejunum, revealing 3 exophytic tumors, provided specimens for analysis. Following histological examination, a diagnosis of IMT was made. A review of the literature regarding this rare disease is also presented to emphasize the risk of local recurrence and the importance of adequate long-term follow-up.