Paragangliomas are rare with multicentricity being more common in patients with familial history. Early recognition of the characteristic symptoms of headache, palpitations, and diaphoresis in a patient with hypertension and prompt appropriate intervention can minimize the morbidity associated with such tumors and prevent a potentially fatal outcome. Paragangliomas represent neoplasms of neural crest origin that arise from paraganglia. Paragangliomas are tumours arising in extra-adrenal paraganglia either in the paravertebral space. We report a rare case of two discrete paragangliomas in the retroperitoneum of a young girl presenting with throbbing headache. Contrast Enhanced CT Abdomen showed the lesions as a large irregular heterogeneous and intensely enhancing mass lesions. Elevated levels of vanillylmandelic acid were found in the Urine. The lesions were removed by surgery and the histopathologic findings of both the lesions confirmed the diagnosis of multiple paragangliomas. Early identification and early surgery will minimize the potential risks and complications in this condition.
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