Adrenocortical carcinoma is an aggressive but rare neoplasm of the adrenal cortex, with an estimated incidence of approximately 2.5 per one million patients. The prognosis for patients with adrenocortical carcinoma is often very poor. Patients often present with symptoms of hormone hypersecretion but may also present with pain or a palpable mass. Imaging plays an important role in preoperative planning when clinical and biochemical findings are compatible with adrenal cortical carcinoma. We report a case of adrenocortical carcinoma in a young woman who presented with classical Cushing syndrome, but who had an atypical hormonal profile.