We report the clinical case of a 48-year-old woman with advanced renal cell carcinoma, and an inferior vena cava thrombus extending into the right atrium. The patient underwent complete tumor excision with radical nephrectomy and inferior vena cava trombectomy using adjunctive cardiopulmonary bypass and deep hypothermic circulatory arrest. Pathological studies revealed no capsular invasion by the renal cell carcinoma. Eighteen months postoperatively the patient is asymptomatic and on immunosuppressive therapy for suspected metastatic disease. We discuss the morbilidity, mortality and long term survival of patients with similar presentation of renal cell carcinoma based in a review of the published literature. Long term survival after surgical treatment is possible in a patient with localized renal cell carcinoma extending into the right atrium. In patient with localized renal cell carcinoma and inferior vena cava tumor thrombus the cephalad extent of inferior vena caval involvement does not appear to influence the prognosis.