The only curative treatment is surgery. The laparoscopic approach is avoided to preserve the integrity of the tumor and prevent rupture of the capsule. Resection should be as complete as possible, including adjacent organs, if affected, nodes, and metastases. Reoperation is indicated in recurrences. Surgery is not indicated if tumoral resection cannot remove > 90% of the tumor or if metastases cannot be eliminated. If there is residual tumor, radiotherapy can be used on the tumoral bed. Moreover, medical treatment is used as coadjuvant to surgery and radiotherapy with the aim of controlling tumoral growth and any hormonal overproduction. The most frequently used medical treatment is mitotane therapy, whose mechanism of action works through its adrenolytic effect and reduction of cortisol synthesis due to 11-beta-hydroxylase blockade. However, this drug has multiple secondary effects and its clinical effectiveness is controversial. Chemotherapy can also be associated and the best results have been obtained with the “Italian protocol”, which combines mitotane, etoposide, doxorubicin, and cisplatin. Other drugs can be used to inhibit steroidogenesis, such as ketoconazole, metopirone, etomidate, and aminoglutethimide. Despite these distinct therapeutic possibilities, the prognosis of patients with this tumor remains extremely poor. Consequently, new drugs aimed at influencing distinct aspects of the biochemistry of ACC are being investigated. However, these drugs currently represent a theoretical possibility, without clinical application.