BackgroundInflammatory myofibroblastic tumor is a rare tumor of a borderline malignancy. Although it is commonly seen in children, the number of childhood cases in the current literature is limited. The lung is the most commonly affected location. However, cases that have been documented in the mesentery-omentum have mostly been located in the mesentery of the small bowel and not in the antimesenteric edge as in our patient.Case presentationA 6-year-old Greek boy was referred to our hospital with acute abdominal pain mimicking appendicitis. Ultrasound and computed tomography revealed a solid mass in the abdomen. The patient underwent laparoscopic resection of the tumor, and histopathology and immunohistochemical analysis favored an omental-mesenteric myxoid hamartoma, which is a variant of an inflammatory myofibroblastic tumor. The patient’s postoperative course was uneventful, and he has been asymptomatic during follow-up.ConclusionsInflammatory myofibroblastic tumor of the small intestine is a rare, benign neoplasm in children that should be considered as a possible cause of acute abdomen. A precise diagnosis can be made on the basis of histologic findings. Surgical excision is the treatment of choice.