Inflammatory myofibroblastic tumor of intestine presenting as intussusception

Inflammatory myofibroblastic tumor (IMFT) is a rare mesenchymal tumor predominantly seen in the lungs. Intestinal IMFT is extremely rare and may cause intussusception. Surgery is the mainstay of treatment as IMFTs are resistant to conventional chemoradiation. Long term follow up is required as there is high recurrence risk. About 50% of IMFTs have translocations involving the anaplastic lymphoma kinase (ALK) gene which has been therapeutically targeted using Crizotinib. We report a 1-year-old boy with ileo-ileal intussusception who was operated after a trial of conservative management. Laparotomy revealed an ileal mass that was resected and histopathology confirmed IMFT. Recovery was uneventful.