OBJECTIVE To report a rare case of a metastatic adrenocortical carcinoma (ACC) that achieve a complete and a long-term remission. METHODS AAC is a rare and aggressive tumor, with a high risk of recurrence and that present metastases in 21% of cases at diagnosis. Treatment of advanced ACC is challenging, mitotane is the only available adrenolytic treatment, with modest and unpredictable responses. Response rates to systemic chemotherapy are not encouraging. We describe the case of a 39-year-old woman with a metastatic ACC, that achieve a complete and long-term remission after chemotherapy, mitotane treatment and surgery of primary tumor and liver metastases. CONCLUSIONS A complete remission of a metastatic adrenocortical carcinoma is possible in some rare cases after a multimodal treatment.