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.