In about 90% of cases tumors arising from chromaffin cells are located in the adrenal medulla and are commonly termed pheochromocytomas, whereas in 10% of cases tumors are extraadrenal and are termed paragangliomas, with 90% of such tumors being intraabdominal. Endothoracic paragangliomas are rare and usually have no symptoms. Incidentally however, the tumors may have neuroendocrine activity, secreting adrenocorticotropic hormone (ACTH) and/or catecholamines. Endothoracic paragangliomas are considered to be of low-grade malignancy. 1 Here, we report a case of thymic paraganglioma presenting with Cushing syndrome.A 39-year-old man presented with a 4-month history of generalized edema, weakness, blurred vision, hypertension, tachycardia, and weight gain of 10 kg. He had experienced development of hyperpigmentation and increased body hair and was able to sleep only 4 to 5 hours a night.His mean 24-hour blood pressure and heart rate values were 179/132 mm Hg and 114 beats per minute, respectively. He was obese and showed cushingoid features, including moon face, facial hirsutism, buffalo hump, hyperpigmentation, lateral abdominal purple stripe (Fig. 1A), and severe muscle weakness of the limbs.Laboratory examination showed the presence of hypernatremia, hypopotassemia, and leukocytosis. The patient's serum concentrations of epinephrine and norepinephrine were within normal ranges. His serum levels of ACTH and cortisol were markedly increased (Table 1). However, cortisol production could not be suppressed after administration of high-dose dexamethasone, and his plasma ACTH level was also increased (to 209.49 pg/mL). A magnetic resonance imaging scan of the pituitary gland was read as normal. Therefore, a diagnosis of Cushing syndrome due to the ectopic secretion of ACTH was affirmed. A computed tomography(CT)scan of the abdomen with contrast showed no evidence of abnormalities, including no adrenal gland abnormalities, but a chest CT scan with contrast revealed an 8 Â 8-mm well-circumscribed ovoid nodule in the thymic region (Fig. 1B).Preoperatively, the b1-blocker metoprolol and a1blocker terazosin were used to control heart rate and blood pressure, respectively. The thymic lesion was assumed to be a thymic carcinoid and was completely resected by video-assisted thoracic surgery; there was no significant lymph node in the chest and mediastinum. The gross appearance was a firm, well-circumscribed but ovoid 8 Â 8 Â 8-mm nodule without a capsule. Figure 1. Thymic paraganglioma. (A) Lateral abdominal purple stripe. (B) Chest computed tomography revealed a 8 Â 8-mm well-circumscribed ovoid nodule (red arrow) in the thymic region. (C and D) The tumor shows an expanding border surrounded by normal thymic tissue. Disclosure: The authors declare no conflict of interest.