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ABSTRACT: Objective: Adrenocortical carcinoma (ACC) is a rare disease often associated with hormonal hypersecretion. Hypercortisolism is the most common endocrine syndrome associated with ACC. Abiraterone acetate is a selective and irreversible inhibitor of cytochrome P450 17A1 that is currently used in the treatment of metastatic castration-resistant prostate cancer. The drug induces a profound suppression of both androgen and cortisol secretion and it is a plausible candidate for medical treatment of Cushing's syndrome. We evaluated the hormonal effects of abiraterone administration in a patient with Cushing's syndrome induced by heavily pre-treated metastatic ACC.Methods: A 50-year-old woman with ACC, suffering from severe and unmanageable Cushing's syndrome despite mitotane therapy, received oral abiraterone at 500 mg/day.Results: After 9 days of treatment, the patient experienced a dramatic improvement in autonomy and deambulation, reduction of edema, better control of blood pressure and glycemia, serum potassium normalization, restoration of the sleep-wake rhythm, and disappearance of mental confusion episodes. Hormonal laboratory tests showed a marked reduction in circulating androgen levels and urinary cortisol excretion (urinary free cortisol levels lowered from 1,863 to 520 μg/24 h). Abiraterone therapy was interrupted due to subsequent deterioration of the patient's condition caused by the progression of ACC.Conclusions: Abiraterone can be an effective medical treatment in the management of Cushing's syndrome induced by ACC. This preliminary observation warrants confirmation in a prospective clinical trial.Abbreviations: ACC = adrenocortical carcinoma