Background: Adrenal myelolipoma is a rare benign neoplasm which is composed of mature adipose tissue and scattered islands of hematopoietic elements. Usually, myelolipoma is small (<4 cm), asymptomatic, and nonsecreting. In some cases, it may cause symptoms such as chronic pain. It can also be hormonally active, as there is a relatively high incidence (10%) of associated endocrine disorders, such as Cushing’s syndrome, Conn’s syndrome, congenital adrenal hyperplasia, adrenal insufficiency, and pheochromocytoma. On the other hand, primary hyperparathyroidism is a common disorder caused by over-activation of parathyroid glands, resulting in the excessive release of the parathyroid hormone. Case Presentation: Herein, we present a female patient who presented with abdominal pain due to adrenal myelolipoma, who was also found to have primary hyperparathyroidism and we discuss the possibility of the coexistence of multiple endocrine neoplasia type 1 syndrome. Conclusion: To our knowledge, this is the first case of a coexistence of an adrenal myelolipoma together with an ectopic parathyroid tissue adenoma. The combination of an adrenal myelolipoma with an ectopic parathyroid tissue adenoma has never been described before and may or not be just a coincidence.

Keywords: Adrenal myelolipoma, primary hyperparathyroidism