Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. The diagnosis of malignancy relies on careful investigations of clinical, biological, and imaging features before surgery and pathological examination after tumor removal.1 We reported a case of a 23-year-old male patient who presented with persistent left flank pain and urticaria for 3 months. Imaging studies confirmed the presence of a large left adrenal mass with malignant features. The biochemical workup was unremarkable. Open left radical adrenalectomy was performed, the final pathologic examination showed ACC with negative surgical margins. The patient remained disease-free for eighteen months period of follow-up after surgery.2