Fibrous or desmoid tumors are grouped under the term fibromatosis; there are several groups; the pediatric aggressive form is rare, especially the pelvis and/or hip presentation. This causes a variable degree of destruction of the surrounding tissues. It is a diagnosis by exclusion and histopathologic testing; treatment is always surgical and conservative treatment is indicated only for special locations and/or patient conditions. This article reports a case of aggressive pediatric hip fibromatosis with severe joint destruction, as well as the work-up and management protocol followed at the National Pediatrics Institute Pediatric Orthopedics Service to approach bone tumors. No cases similar to this one have been reported.

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