The study presented a rare case of inflammatory myofibroblastic tumor (IMT) in a 57-year old male patient who presented with recurrent abdominal pain. He was diagnosed to have a tumor of the small intestine within ileal intussusception, demonstrated by CT enterography. The patient underwent surgery to remove the tumor with a margin of healthy tissue. Histopathological and immunohistochemical examination results enabled to diagnose IMT. IMT is a rare tumor that occurs mainly in children and young adults. Its etiology remains to be fully understood. Due to the differentiated histology it can be found in many organs and soft tissues, being responsible for different, non-specific clinical and radiological symptoms. Due to the rarity of this tumor a clear treatment protocol has not yet been established. However, given the tendency to recur with possible distant metastases an important element of treatment consists in long-term clinical patient observation.