Leiomyosarcoma (LMS) are rare tumor associated with poor outcomes. Treatment of the condition is challenging due to limited cases in the literature. Here, we present the case of a 46-year-old male who presented with left lower limb deep vein thrombosis, with an ultrasound scan revealing right renal mass. MRI demonstrated a large, wellcircumscribed lesion engulfing in the middle and lower right renal poles associated with an invasion of the related part of the IVC, right renal vein and mid-length of the left renal vein. The patient underwent right radical nephrectomy and IVC thrombectomy. Subsequent pathology was consistent with an LMS of the inferior vena cava. We document this case and discuss implications.