Rationale:The infantile fibrosarcoma (IFS) is a non-rhabdomyosarcoma soft tissue sarcoma with locally aggressive properties. State of the art therapy consists of neoadjuvant chemotherapy followed by wide resection according to the criteria of the musculoskeletal tumor society.Diagnoses:An ETV6-NTRK3 positive IFS of the distal tibia in a 21-months old child showed good response to chemotherapy.Interventions:Due to refusal of amputation marginal resection completing the margins with a high speed drill and filling the space with bone cement was performed.Outcomes:At latest follow-up 10 years after surgery, no recurrence was observed.Lessons:An individual therapy for surgical treatment of IIFS is recommended. This comprises marginal resection in instead of the golden standard “wide resection” in selected cases.