Although preoperative clinical, biochemical, and radiologic features can aid in the diagnosis of adrenocortical cancer (ACC), uncertainty often remains. This diagnostic ambiguity and the subsequent potential for an inadequate surgical resection have likely contributed to the currently dismal disease-free survival, although unsettlingly high rates of locoregional recurrences still persist even in the setting of a supposedly R0 resection. Refinements in both diagnostic criteria and surgical techniques, as well as the increasing use and study of novel multimodality therapies for ACC, have provided advances in the treatment of these patients, and renewed hope for meaningful improvements in patient outcomes.