To report a case of paraganglioma localized in the organ of Zuckerkandl and to discuss its diagnosis, treatment and outcome.A 37-year-old female presented with a periumbilical mass and pain that radiated to the lumbar region for the past three months. Abdominal US and CT disclosed a well vascularized mass lying adjacent to the aorta, 1 cm from its bifurcation. At laparotomy, an 8 x 10 cm tumor was discovered at the aortic bifurcation that displaced the left ureter and included the inferior mesenteric artery. Pathological analysis of the surgical specimen revealed a paraganglioma.Extra-adrenal paraganglioma should be taken into account in the differential diagnosis of retroperitoneal masses, particularly those adjoining the abdominal aorta.