In reviewing the experience of a number of authors and investigators, it is clear that early diagnosis of adrenocortical carcinoma is essential for cure. Of all the modalities of therapy currently available, surgical resection holds the most promise for cure or prolonged survival. Treatment for extensive local disease or metastatic disease has been discouraging, and the prognosis for reasonable, comfortable survival is poor. Unfortunately, the toxicity of mitotane, an adrenolytic agent and currently the most effective drug available, is often unacceptable and may militate against its use. Because many of the debilitating side effects of these tumors are related to hormone production, newer drugs that result in hormonal blockade may add considerably to the comfort of the patient. The development of less toxic chemotherapeutic agents presents a challenge for both the oncologist and the endocrinologist.