We present an unusual case of a 41-year-old woman with a known glomus tumor, an adrenal mass, hypertension, and elevated catecholamines. The glomus tumor was shown to be the site of excessive catecholamine production in what we believe to be one of the few descriptions of 123I-metaiodobenzylguanidine (MIBG) scanning for this uncommon tumor. The diagnostic difficulties of such a case are discussed. A literature review of catecholamine-secreting glomus tumors and a systematic approach to catecholamine-secreting tumor localization in such patients is presented. Therapeutic options of surgery, radiation therapy, and embolization are reviewed. We conclude that the management of patients with functioning glomus tumors needs to be individualized. A careful, systematic approach is required if needless surgery is to be avoided. Further, the use of 123I-MIBG scanning deserves consideration to help localize catecholamine production in such patients.