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Adrenocortical carcinoma is a rare, aggressive cancer with a poor prognosis. Management of early-stage disease relies on surgical resection, which remains the only potentially curative therapy. Open surgery remains the standard of care, with minimally invasive approaches being appropriate in cases with smaller tumors and no evidence of local invasion. Advanced disease traditionally relies on mitotane-based chemotherapies to control disease progression. Clinical trials evaluating the potential efficacy of immunotherapy, checkpoint inhibitors, and tyrosine kinase inhibitors are ongoing but have yet to yield results that impact guideline-based therapies. Other therapies, such as radiation, are commonly used for recurrent or metastatic disease.