A 40-year-old Japanese female was referred to our institution with a high serum lactate dehydrogenase level. Computed tomography (CT) showed a large right adrenal tumor, 14 cm in size without distant metastases. The patient was clinically diagnosed with T2N0M0 adrenocortical carcinoma and underwent right adrenalectomy. The pathological diagnosis was adrenocortical carcinoma with negative surgical margin. The patient was administered mitotane for 2 years as adjuvant therapy. Subsequently, CT revealed asynchronous multiple metastases, including liver, lung, left kidney, and right acetabulum. The patient received 15 courses of EDP (a combination of etoposide, doxorubicin, and cisplatin) plus mitotane therapy, and had stable disease without new lesions.