Steroid hormones have been implicated in the growth and/or development of meningiomas. A 46, XX male with untreated congenital adrenal hyperplasia who developed meningiomas is presented here. The meningiomas only expressed progesterone receptors, as most meningiomas do. The high 17-hydroxyprogesterone levels, resulting from his disease, could have played a role in the development and growth of his meningiomas.