ABSTRACT
Carney Complex is an autosomal dominant syndrome that is defined with different tumors including myxoma in different organs, endocrine tumors and lentiginosis lesions. This is the first case report of this syndrome from Iran. Case: The patient is a 27 year old girl, referred with flank pain. Physical examination revealed hirsutism, truncal obesity, hyperpigmantasion and hypertension; Cushing's syndrome was suggested and confirmed with related classic biochemical tests. She had history of cardiac myxoma during her childhood and had been operated twice. Pituitary microadenoma and right adrenal adenoma were reported on MRI and CT-scan, respectively. Initially laparascopic right adrenalectomy was done; as expected, no remission in signs of Cushing's syndrome was observed after surgery. By laparoscopic adrenalectomy of the other site, Cushing's syndrome resolved. Pathologic report of 1st operation was adrenal adenoma with surrounding pigmented micronodular hyperplasia and of the 2nd one was just pigmented micronodular hyperplasia. Conclusion: We have reported a patient with Carney syndrome along with Cushing's syndrome due to bilateral adrenal hyperplasia and an adenoma in contralateral adrenal and microadenoma of the pituitary as an incidentaloma. This is a new presentation of Carney syndrome