In summary, we reported the first case of primary pancreas NTRK-rearranged spindle cell tumor with a special sclerosing epithelioid fibrosarcoma pattern harboring EVT6::NTRK3 gene fusions, CDKN2A/2B homozygous deletion, and ARID1A mutation. This evidence concerns the gene NTRK3 and spindle cell neoplasm.