Myelolipoma is an uncommon benign tumor of unknown etiology and adrenal myelolipoma is rarely associated with endocrine disorders. We report a 67-year-old woman with Cushing's syndrome due to left adrenal adenoma associated with myelolipoma. The patient underwent laparoscopic left adrenalectomy and pathological examination revealed an adrenocortical adenoma associated with myelolipoma. To the best of our knowledge, 25 cases of endocrine dysfunction associated with myelolipoma have been reported in the English and Japanese literature. We review and discuss the pathogenesis of adrenal myelolipoma.