(123)I-metaiodobenzylguanidine (MIBG), a radio-labeled catecholamine analogue, is used for the imaging of pheochromocytoma based on the selective uptake of MIBG by chromaffin tissues. MIBG scintigraphy displays high sensitivity (90%) and specificity (close to 100%). In contrast, the false-positive uptake of MIBG by adrenal cortical carcinoma is rare. Here, we report a metastatic oncocytic adrenal cortical carcinoma with MIBG uptake used for therapeutic purposes.