Adrenal myelolipomas are rare benign tumors that comprise mature adipose cells and hematopoietic elements. They are usually found incidentally at autopsy and on imaging. Most cases are isolated and small. Although they are not hormonally active, there is very rarely an association with functional adrenal disorders such as Cushing syndrome and congenital adrenal hyperplasia. It is believed that in these patients high corticotropin levels contribute to the pathogenesis of these neoplasms. We report the imaging appearances of bilateral giant adrenal myelolipomas in a patient known to have congenital adrenal hyperplasia. To our knowledge, these are the largest bilateral adrenal myelolipomas reported in association with congenital adrenal hyperplasia. A distinct change in their appearances after a period of steroid treatment is described for the first time.