An extra-adrenal paraganglioma is a rare tumour derived from chromaffin cells of sympathetic ganglia. This report documents a rare case of a non-functional aortocaval paraganglioma in a 24-year-old woman with persistent abdominal pain. Computed tomography revealed a solid mass, measuring 2.5x3cms, localized between the celiac trunk and superior mesenteric artery in aortocaval location along with right ovarian cystic mass. A clinical diagnosis of malignant ovarian tumour with celiac nodal metastasis was made. Excision and pathological analysis of both revealed an aortocaval extra-adrenal paraganglioma and benign ovarian cyst. On serial follow-up the patient was in a good health, asymptomatic and without evidence of tumour recurrence. This case emphasizes the necessity of including extra-adrenal paraganglioma in the differential diagnosis and management of retroperitoneal tumours, despite its rarity.
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