Pediatric fibroblastic-myofibroblastic tumors of intermediate prognosis are locally aggressive tumors that rarely metastasize. They are potentially curable, but managing them is often a challenge in terms of their correct diagnosis and appropriate treatment. This paper reviews the most recent biological findings and latest novelties in the multidisciplinary treatment of childhood desmoid-type fibromatoses, infantile fibrosarcoma and inflammatory myofibroblastic tumor.