Adrenocortical carcinoma is a rare endocrine tumor in childhood. It is curable when it is resected totally. However, medical therapy is challenging in patients with metastatic disease. Herein, we present the response to various salvage chemotherapy modalities in addition to mitotane therapy, which were administered to a child with progressive and metastatic adrenocortical tumor. The patient has been under follow-up with progressive disease for 17 months.