Adrenocortical carcinoma (ACC) is a rare and highly malignant neoplasm. We present the case of a 51-year-old male patient with a left-sided ACC admitted to hospital with ipsilateral flank pain, weight loss, difficulty in breathing, abdominal discomfort and swelling and bilateral leg edema. Thoracoabdominal computed tomography revealed a huge adrenal mass with obvious tumor thrombus involvement of the inferior vena cava and right atrium. This is the first report describing caval and opposite side renal vein invasion of a left-sided ACC treated with grafting of the vessels. Histopathological examination of the tumors confirmed the diagnosis of ACC. Postoperative recovery was uneventful. The patient received an adrenolytic agent, mitotane, postoperatively and is alive with no evidence of recurrence after 2 years of follow-up. Copyright (c) 2008 S. Karger AG, Basel.