We report and describe the presentation, pathological diagnosis with immunostaining and management of a rare case of capsular renal leiomyosarcoma encasing the inferior vena cava (IVC). We have reviewed and tabulated other such similar cases. The present case was successfully managed by radical nephrectomy and adjuvant radio-chemotherapy. Immunostaining should be freely used to define the histological type of renal sarcoma in order to accurately counsel and deliver a prognosis for patients with renal leiomyosarcomas with a poor prognosis.