We report a rare case of a solitary primary paraganglioma arising in the mesentery, found in a 72-year-old woman who presented with abdominal pain and a palpable abdominal mass. This extra-abdominal paraganglioma developed from paraganglionic cells that travelled by vertebral migration from the root of the superior mesenteric artery. Extra-adrenal paraganglia extend anywhere from the neck down to the base of the pelvis. Ultrasonography, computed tomography (CT), and angiography showed a solid and cystic heterogeneously enhanced mass, which was fed by the superior mesenteric artery, without distant metastasis. Exploratory laparotomy revealed a large, dark, brownish-red mass in the mesentery of the ileum, which was distinct from the ileum. The mass consisted of peripherally solid areas with central hemorrhage and cystic degeneration. It was diagnosed as a paraganglioma histologically. The patient is free from recurrence of paraganglioma after 1 year of follow up. To our knowledge, this represents only the seventh case of a paraganglioma arising in the mesentery.

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