The patient is well and continues treatment.<h4>Conclusion</h4>The clinical and radiological response of our patient with an uncommon TPM4 (exon 7)-NTRK1 (exon 12) gene fusion tumor treated with a first-generation TRK inhibitor could contribute to a better understanding of the biology of this new STS entity and help to improve patient management. The gene discussed is NTRK1; the disease is telomere syndrome.