摘要篇首：A denomatoid tumors (ATs) are benign neoplasms that most commonly occur in the genital tracts of both male and females.~1 Extragenital ATs are rare and have been reported in the pleura, heart, omentum, the mesentery of the small intestine, pancreas, mediastinal lymph nodes and peri-umbilical skin.~(1,2) AT of the adrenal gland is typically an asymptomatic neoplasm with benign behavior, which has been recently recognized as a diagnostic challenge, as it is both rare and can present in such a manner as to suggest a wide range of differential diagnoses.1'3'8 Here we report a very large AT of the left adrenal gland, which at present appears to be the largest tumor of this type that has ever been reported. It may be the first case of AT associated with a high level of plasma adrenocorticotropic hormone (ACTH).