Extra-adrenal paragangliomas (PGOMAs) are rare and can occur anywhere along the sympathetic chain including the carotid body, glomus jugulare, vagal bodies, ganglium tympanicum, larynx, ciliary bodies, organs of Zuckerkandl, urinary bladder, and other locations. These are microscopically identical. The embryological origin is from neural crest cells. One to three percent are reported to be functioning, predominantly secreting norepinephrine. Diagnosis of a functioning PGOMA can be delayed, even when symptoms of catecholamine hypersecretion are present. Furthermore, diagnostic testing and surgery have a significant morbidity if the diagnosis is not considered in advance, as illustrated by the following cases. PGOMAs can be sporadic or familial with an autosomal dominant pattern with variable penetrance. They can be multicentric and associated with other endocrine gland tumors. Benign and malignant PGOMAs have been described, with malignancy being defined by lymph node metastasis. Few cases of functioning glomus jugulare and carotid body tumors have been reported in the medical literature.