Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. Surgery is the only curative therapy available and overall 5-year survival for patients who undergo a complete resection is 32% to 48%. They are known to produce intravascular invasion and into the inferior vena cava (IVC) and in rare cases they may reach the right atrium. We report a case of functioning ACC extending into the inferior vena cava and right atrium in a female with Cushing's syndrome.