We report a case of a compound adrenal medullary tumor. A 63-year-old woman was referred to our hospital for examination of a right adrenal tumor, incidentally found by abdominal computed tomography (CT). CT and magnetic resonance imaging (MRI) revealed a round heterogeneous tumor, 5 cm in diameter, on the upper pole of the right kidney. A view of the total body scan demonstrated the uptake into the tumor after the injection of 123I-metaiodobenzyl-guanidine. Serum and urinary adrenaline levels were slightly elevated, and urinary excretion of vanillylmandelic acid was markedly elevated. Her blood pressure was normal. From these findings, the tumor was suspected to be a pheochromocytoma of the right adrenal gland and was resected reteroperitoneally. Pathological diagnosis was a compound adrenal medullary tumor, which was composed of pheochromocytoma and ganglioneuroma. This combination of the adrenal medullary tumor is extremely rare, and to date this case may be the sixth case in the Japanese medical literature.