We present our experience in the management of children with inflammatory myofibroblastic tumor, a total of five children with two presenting with superior mediastinal syndrome and three with abdominal disease. We treated all children with a low-dose oral chemotherapy protocol, including prednisolone, 6-mercaptopurine, methotrexate, and celecoxib. Children with the mediastinal disease showed complete response with chemotherapy alone, while the child with sigmoid polyp is in remission with the chemotherapy post complete excision. We commenced two children with abdominal disease on crizotinib due to local recurrence with an adequate response. Long term follow-up and prolonged therapy are essential to sustain remission.