Purpose <p>Low-grade myofibroblastic sarcoma (LGMS) is a rare fibroblastic/myofibroblastic malignancy, predominantly occurring in the head and neck and generally exhibits limited sensitivity to radiotherapy and conventional chemotherapy. Only sporadic cases of spinal LGMS have been reported. The aim of this study was to gain a better understanding of the distinctive features of spinal LGMS and explore its treatment options through retrospective analysis of 7 consecutive patients with spinal LGMS in the context of a literature review.</p> Methods <p>The clinical data and prognosis of 7 patients who received surgical treatment at our center between March 2019 and December 2024 were analyzed retrospectively.</p> Results <p>The 7 patients included 2 males and 5 females ranging in age from 24 to 71 years, with a mean age of 42.6 years. The tumors involved the cervical vertebra (<i>n</i> = 1), thoracic vertebra (<i>n</i> = 1), lumbar vertebra (<i>n</i> = 1), and sacrum (<i>n</i> = 4). The mean intraoperative blood loss was 2100 ± 1322.9&#xa0;ml, the mean operation time was 361.9 ± 152.9&#xa0;min, and the mean follow-up duration was (19.4 ± 11.7) months. Four of the 7 patients died during the follow-up period despite postoperative adjuvant therapy, including 3 patients who died of in-situ recurrence with distant metastasis, and one patient who died of in-situ recurrence. The remaining 3 patients survived with no sign of disease at the last follow-up visit.</p> Conclusion <p>The prognosis of spinal LGMS is usually poor. Although evidence suggests that spinal LGMS exhibits limited sensitivity to radiotherapy and chemotherapy, surgical resection is typically pursued as the main treatment modality following pathological confirmation, while recognizing the need for individualized clinical decision-making. Factors affecting the prognosis of spinal LGMS include tumor size, tumor resection methods, tumor location, and the complex anatomies of the spinal cord.</p>