ACC is a rare clinical entity that carries a poor prognosis; early diagnosis and complete surgical resection are associated with the improvement in patient survival. Even with appropriated diagnosis and treatment, most patients will develop recurrence and succumb to ACC because of the underlying tumor biology, the difficulty of achieving a complete resection, and the lack of effective systemic therapies. Despite its many drawbacks, mitotane continues to be a mainstay in the treatment of high-risk patients with ACC, especially those with recurrent or metastatic disease. Recent findings suggest that mitotane, combined with conventional chemotherapeutic agents, may improve survival for such patients.