Paragangliomas are non-epithelial tumours originating from neural crest-­derived paraganglion cells situated in the region of the autonomic nervous system ganglia and accompanying nerves. The paraganglion system can be divided into the adrenal medulla and the extra­adrenal paraganglion system. The latter can be further subdivided into the para­sympathetic and sympathetic paraganglia. The parasympathetic paraganglia are primarily located in the head and neck and are less frequently located in the thorax and pelvis. The sympathetic paraganglia are primarily located along the sympathetic nerve chains bordering the vertebrae in the abdomen and pelvis.