Bilateral adrenal myelolipoma associated with adrenogenital syndrome

Adrenal myelolipoma is a rare benign tumor, occasionally reported in association with endocrine disorders. We report herein a case of bilateral adrenal myelolipoma associated with adrenogenital syndrome caused by 21-hydroxylase deficiency. A diagnosis of 21-hydroxylase deficiency was confirmed by mutation analysis of the CYP21 gene. Our case represents only the second case of bilateral adrenal myelolipoma associated with adrenogenital syndrome caused by 21-hydroxylase deficiency.