Primary vertebral leiomyosarcoma of the spine is extremely rare. Due to its rarity, the number of studies on
pathology, treatment and prognosis is limited. Tissue Biopsy and Immunohistochemical staining techniques
provide unequivocal diagnosis of leiomyosarcoma. The mainstay of treatment of spinal leiomyosarcoma is
surgery, with or without radiotherapy and embolization. The principles of surgery comprise resection of
tumor, decompression and stabilisation of the spinal cord and prevention of tumor recurrence. The Tomita
total en-bloc resection is the preferred surgical technique. We hereby present a literature review of spinal
leiomyosarcoma and a case report of the first case of a 56-year-old lady in Malaysia who was diagnosed
with L3 vertebral leiomyosarcoma and lung metastasis.