Extra-adrenal abdominal paragangliomas are rare. Most arise from the organs of Zuckerkandl, involve large concentrations of paraganglionic tissue, and are located in the para-aortic space along the sympathetic chain. Published reports have, however, described normal paraganglionic tissue at the root of the mesentery which serves as the superior limit of the organs of Zuckerkandl, and mesenteric paraganglioma is very rare. We report a case of paraganglioma with cystic degeneration arising from the mesentery.