ABSTRACT Objective: Primary leiomyosarcoma of bone (PLB) is a rare type of malignant bone tumor considered as a variant of the spindle cell sarcomas (SCS). The objective of this study was to analyze the clinicopathologic and the prognostic factors of patients with PLB treated at a single institution. Methods: We retrospectively reviewed the records of 22 patients with pathologically confirmed PLB. The data collected were: age, sex, tumor size and location, grade and stage of the disease and histopathologic features. Mean age was 45.5 years (range, 17 to 73 y). Location was: upper limb (27.3%), lower limb (68.2%) and pelvis (4.5%). Patients had high grade in 90.9% of the reports. Margins were negative in 77.3% of the cases. Histological reports describe spindly sarcomatous cells arranged in fascicles with increased vascular formation without osteoid or chondroid matrix production. On immunohistochemistry, smooth muscle actin and desmin where positive in all cases. Results: Mean follow-up time was 73.5 months (range, 5.3 to 331.1 m). We found 22.7% of local recurrence (LR). Distant metastasis (DM) was reported in 9 (40.9%) patients. Lung metastasis was the only DM affected site. Overall survival (OS) rate in 5 years was 59.1%. Predictors of OS were LR and DM. Conclusions: PLB is an extremely rare malignant bone tumor that has a higher rate of DM and similar OS prognosis compared with other bone sarcomas. Level of Evidence IV, Case Series.