A 6-year-old female presenting with an abdominal mass was found to have an unresectable undifferentiated sarcoma...molecular testing identified an NTRK3-fusion, and treatment was changed to larotrectinib monotherapy....This drug was administered at a dose of 100 mg/m2 twice a day. A CT scan performed after 3 months of therapy demonstrated a very good partial response with a 95% decrease in tumor size, based on RECIST 1.1 criteria....The patient was maintained on larotrectinib, and 3 months after completing radiotherapy a repeat CT scan showed further minor reduction in the size of the tumor.... Larotrectinib was continued for 12 months following completion of radiotherapy, for a total of 22 months. The patient remains in remission 9 months after stopping all anticancer treatment.