Adrenocortical carcinoma (ACC) is a rare cancer with a generally poor prognosis. In approximately 60% of cases the initial clinical manifestations are due to hypersecretion of adrenocortical hormones. Cushing's syndrome is the most frequent hormonal manifestation. In the remainder of the cases the tumor is identified after imaging procedures for nonspecific complaints such as abdominal pain and nausea. Medical therapy is employed in patients with unresectable or partially resected tumor and metastatic disease. The current accepted treatment is a combination of Mitotane with chemotherapy, aimed at controlling hormonal hypersecretion and reduction of tumor mass. In ACC patients there is wide variability in the course of the disease: some with metastatic disease will survive for more than 10 years, others succumb to the disease within months.