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Tumor thrombus formation is a unique aspect of renal cell carcinoma with significant therapeutic and prognostic implications. The prognostic significance of cephalad extent of tumor thrombi to the right atrium remains controversial. Extended surgical removal, however, is the only way to expect survival. In 1989, a 40-year-old man was diagnosed with an advanced renal cell carcinoma (T3CN2M0) involving perinephric fat, hilar and para-aortic lymph nodes and a tumor thrombus extending to the right atrium. He was treated with extensive surgical resection of the tumor and its lymphatic and vascular extensions. Interferon-α injections were given for 2.5 years as an adjuvant immunotherapy. The patient was annually checked with abdominal ultrasound, chest X-ray and computed tomography, but has manifested no local or distant metastasis for 18 years. To our knowledge, this is the first reported case of extensive surgery on advanced renal cell carcinoma with no evidence of recurrence for 18 years.