RESUMO
Adrenocortical carcinoma (ACC) is a rare malignancy, especially in children. The overall incidence is approximately 2 cases per million per year.[1] In children, the incidence is 0.3 cases per million per year, except in southern Brazil where the incidence is 3.4-4.2 cases per million per year.[2] We describe a giant nonfunctioning metastasized ACC in a 6-year-old girl who presented with a history of increasing abdominal girth incidentally noticed by her mother since 1 week. Ultrasound abdomen showed a large right suprarenal tumor with calcifications and necrosis. Empty left renal fossa and compensatory enlarged right kidney were seen. Computed tomography (CT) scan revealed a large heterogenously enhancing right suprarenal mass with calcification and necrosis with pulmonary metastasis. Histopathology report from the right suprarenal mass revealed an ACC. With a stage IV disease, the patient died after 2 months from diagnosis.