1.1. Background: Adrenocortical carcinoma (ACC) in childhood represent about 0.2% of all pediatric malignancies. Children had a higher incidence of virilization in 72% as common presenting symptom. The prognosis in children who have ACC appears to be better than that of adults, at least for early stage disease. The aim of this study was to evaluate the case of child with a low grade of ACC and to confirm the role of the margins free resection in ACC prognostic factors.