Advances of neuroimaging, skull base technique and embolization improved outcome in patients who present with tumor of the glomus jugulare. Catecholamine secreting subgroup, however, is considered to be extremely high risk because of potentially serious complication of an intra- and perioperative hypertension crisis. The authors present detailed description of treatment strategies and perioperative management with a catecholamine secreting glomus jugulare. A 57-year-old woman, in whom the noradrenaline level in plasma was twenty times higher than normal, presented with uncontrolled labile hypertension and carcinoid syndrome. Magnetic resonance imaging (MRI) and 123I-meta-iodobenzylguanidine (MIBG) scan depicted the tumor as originating from the jugular foramen extending to the infratemporal fossa. Alpha catecholamine blocker and magnesium sulfate treatment was commenced prior to embolization and surgery. Under cranial nerve and hemodynamic monitoring, tumor resection via the infratemporal fossa type A was performed. The patient remained hemodynamically stable and the lower cranial nerve injury was able to be avoided. The plasma noradrenaline level decreased and hypertension became normalized. We emphasize treatment strategy, intra- and perioperative management of this rare entity.