In the case report, we present a case of a young adult female patient with soft tissue sarcoma...Molecular diagnostic method confirmed the translocation of a very rare driver oncology target, the neurotrophic tropomyosin receptor tyrosine kinase 3 gene. We used convenient and safe inhibitor of tropomyosin receptor tyrosine kinase larotrectinib therapy with good efficacy and excellent quality of life. Larotrectinib was the first and only systemic therapy to which this metastatic soft tissue tumor responded.