Abstract.A 55-year-old female was admitted to our hospital with severe hypertension (274/140 mmHg). Endocrinological examination revealed that her plasma levels of norepinephrine (NE) was elevated with high levels of urinary NE, normetanephrine and vanillylmandelic acid (VMA), suggesting the presence of pheochromocytoma. However, neither computed tomography nor MIBG scintigraphy detected any catecholamine-producing tumor in or outside the adrenal glands. She was screened with full polysomnography because of heavy snoring, and the diagnosis of severe obstructive sleep apnea syndrome (OSAS) was made. She was treated with calcium channel blocker for three weeks, but severe hypertension persisted. After treatment with nasal continuous positive airway pressure (CPAP) was added, her blood pressure gradually lowered week by week. Concomitantly, the levels of plasma and urinary NE, urinary normetanephrine and urinary VMA were normalized following nasal CPAP therapy for 2 weeks. Additional treatments with alphaadrenergic blocker further decreased her home blood pressure. After a year, she continued nasal CPAP therapy and her blood pressure was nearly below 160/100 mmHg. Urinary NE level was slightly above normal range and other catecholamines stayed within the normal range. This case shows that patients with OSAS could develop severe hypertension through elevated sympathetic tone, mimicking pheochromocytoma. Nasal CPAP therapy is recommended not only to improve hypertension and catecholamine excess but also to distinguish the condition from pheochromocytoma. THERE is a growing body of evidence showing that patients with obstructive sleep apnea syndrome (OSAS) have a high prevalence of hypertension [1][2][3] and that treatment with nasal positive airway pressure (CPAP) successfully lower blood pressure [4][5][6][7]. A major cause of hypertension in OSAS is thought to be the increased sympathetic activity due to apnea-related episodic hypoxemia and repeated arousal [8,9]. There are a number of studies demonstrating the elevated plasma or urinary norepinephrine (NE) or epinephrine (E) levels during sleep and wakefulness in OSAS [10][11][12]. Although it has been reported that nasal CPAP therapy reduces sympathetic activity [10,[13][14][15], the detailed analysis of catecholamine metabolites and the detailed time course of biochemical normalization during nasal CPAP therapy have not been fully examined. Recently, Hoy et al. reported 5 cases of OSAS with drug-resistant hypertension [16]. Catecholaminesecreting tumor could not be identified in any of these subjects, despite high urinary NE levels. Furthermore, treatment of OSAS with nasal CPAP led to normalization of both systemic blood pressure and urinary NE levels. The authors have proposed that pseudopheochromocytoma is a rare, but treatable, presentation of OSAS. Here, we present a similar rare case of the severe hypertensive OSAS patient showing high plasma and urinary catecholamine levels without evidence of catecholamine-secreting tumor. We examined the detailed profil...