Neurotrophic tyrosine receptor kinase (NTRK) rearranged spindle cell neoplasm is an emerging group of molecularly defined rare soft tissue tumors, often presenting with a monotonous spindle cell morphology, infiltrative growth, and co-expression of S-100 and CD34 proteins by immunohistochemistry (IHC). Accurate diagnosis necessitates the combination of morphology, immunohistochemistry, and molecular test results, with next-generation sequencing (NGS) as the gold standard. We present a rare case of NTRK rearranged spindle cell neoplasm of the uterine cervix and review the literature to highlight the current understanding of the diagnosis and treatment of this rare disease. A 49-year-old perimenopausal woman presented with menorrhagia for more than a month. A biopsy of the cervix revealed a cervical spindle cell neoplasm with a tendency to be an isolated fibrous tumor. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed and the surgical pathology suggested NTRK rearranged spindle cell neoplasm, while NGS confirmed TFG-NTRK3 fusion gene. Postoperatively, the patient refused larotrectinib maintenance therapy for economic reasons and had no sign of recurrence or metastasis at 31 months of follow-up. We presented the first case of cervical spindle cell neoplasm with TFG-NTRK3 gene rearrangement and retrieved 22 cases of NTRK rearranged spindle cell neoplasm of the uterine cervix from literature. The most prevalent type of gene fusion was TPM3-NTRK1, and almost all cases demonstrated S-100 and CD34 positivity by IHC. Surgery remains the initial treatment of choice and tyrosine receptor kinase (TRK) inhibitors may serve as a promising target therapy for patients with recurred or metastatic disease.