LMNA::NTRK1-rearranged spindle cell neoplasm is a rare, molecularly defined soft tissue tumor. In the case of a 23-year-old woman, the tumor was characterized by pigmentation, multiple local recurrences and malignant transformation similar to fibrosarcoma - these features have not been described in the literature so far. The neoplasm was diagnosed by integrated analysis of histopathological, immunohistochemical and molecular results. The tumor was positive for the LMNA::NTRK1 fusion, which is both a diagnostic and therapeutic biomarker. In high-risk cases, close monitoring and consideration of targeted treatment with TRK inhibitors is recommended. This case expands the morphologic and clinical spectrum of tumors with LMNA::NTRK1 rearrangements.