Mesenteric paragangliomas are a rare entity; consequently, only 12 cases have been reported to date. Although considered benign and often found incidentally, they have the potential to metastasize and are an important diagnosis to consider for patients with a mesenteric tumor. We report the case of a 71-year-old woman who was found on magnetic resonance imaging to have an incidental, large, complex, intra-abdominal mass that had initially been misdiagnosed as ovarian in origin. She underwent an open resection of the tumor and adjacent small bowel with no perioperative complications; histology confirmed the mass to be a mesenteric paraganglioma. This case report highlights several important key issues regarding paraganglioma, including diagnosis, imaging, genetic testing, and surgical resection. The results of a literature review are also discussed.

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