STUDY DESIGN A primary leiomyosarcoma in the L2 vertebral body without any neurologic deficit is reported. The radiologic diagnosis was a metastasis, and the histologic diagnosis was confirmed by transpedicular needle biopsy. OBJECTIVE To emphasize the occurrence of primary leiomyosarcoma in the lumbar spine. SUMMARY OF BACKGROUND DATA Leiomyosarcoma is a rare malignant neoplasm of the bone, and only one report of the primary spinal tumor exists. In the case of leiomyosarcoma of the bone, metastasis, most commonly of the uterus or gastrointestinal tract, must be carefully ruled out before the diagnosis of primary leiomyosarcoma in the bone is confirmed. METHODS Leiomyosarcoma of the second lumbar vertebra in a 47-year-old woman with no neurologic deficit is reported. Imaging findings indicated a nonspecific high-grade lesion. A total spondylectomy of L2 with en bloc excision of the lower half of L1 and the upper third of L3 vertebral bodies was performed after preoperative radiation therapy. The defect of the vertebral body was reconstructed by titanium mesh cage, and T11 to S1 vertebrae were instrumented by the pedicle screw and rod system. RESULTS The surgical margin was marginal. Histologically, the tumor cells were elongated, with abundant cytoplasm and cigar-shaped nuclei, showing proliferation in fascicles and bundles. No sign of local recurrence or metastasis was evident 2 years and 1 month after surgery. CONCLUSIONS Leiomyosarcoma should be included in the differential diagnosis of spindle cell tumor of the spine, and it should be confirmed or excluded by immunohistochemical and/or ultrastructural studies. If the expected surgical margin is definitive, a total en bloc spondylectomy should be performed.