A case of adrenal carcinoma with the tumor thrombus extending into the right atrium and right ventricle is reported. A 46-year-old man was admitted because of abdominal distention and back pain. Angiogram, venacavography, dynamic CT, cardio-echogram and determinations of blood concentration of hormones disclosed a huge non-functional right adrenal tumor and its thrombus having extended into the right atrium and ventricle via the right adrenal vein. Right adrenalectomy and nephrectomy were followed by simultaneous removal of the tumor thrombus. The resected tumor was 14 X 11 X 7 cm in size and 880 g. The thrombus was 90 g. Pathohistological diagnosis of sarcomatoid adrenocortical carcinoma was made. The patient was discharged three weeks after operation and had been remained asymptomatic for 5 months. He died of the recurrence of the tumor on the 206th postoperative day. In the review of English and Japanese literature, 61 cases of malignant tumors extending into the right atrium were analyzed, among which only one case was an adrenal tumor. From this survey, it seems that aggressive resection would provide better survival, and a surgical excision with a use of cardiopulmonary bypass is worth trying for such conditions.