BACKGROUND: Leiomyosarcoma (LMS) is a rare malignant tumor of smooth muscle origin that generally stems from soft tissues and uterine tissue. However, a small percentage of this sarcoma subset may originate from the smooth muscle of vessel walls, most of which are of venous origin. Although the vena cava (VC) serves as the most likely source for these tumors and has been well described in the literature, there is limited information focused on non-VC LMS derived from large veins. The focus of this study was to consolidate the reports and previously published data of all non-VC LMS of venous origin to better characterize and describe this disease process. METHODS: We reviewed information derived from 143 previously published cases. RESULTS AND CONCLUSIONS: It was determined that women aged 60-69 years were most commonly diagnosed with this particular type of tumor, with the most common tumor site being the renal vein. Metastasis was present in 12% of this population at the time of diagnosis, and 32% of patients were alive 4 years later.