Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. ACC is capable of secreting excess adrenocortical hormones, which can compound morbidity and compromise clinical outcomes. By the time most ACCs are diagnosed, there is usually locoregional or metastatic disease. Surgery is the most important treatment to offer possibility of cure or prolong survival. Several adjuvant therapies are used depending on grade and stage of the tumor and other patient-related factors. This review provides an overview of treatment approaches for ACC, highlighting evidence to support each treatment and acknowledging where more data and research are needed to improve care.