Introduction. Collections of neuroendocrine cells dispersed throughout the body are known as paraganglia and the tumor arising from these paraganglia are known as paragangliomas. Paragangliomas located along side the aorta are associated with the pheochromocytoma and they secrete and store catecholamines. Case report. We reported a 29-year-old woman with episodes of headache, palpitations, dizziness and sweats, associated with hypertension. Elevated urine catecholamines were consistent with pheochromocytoma. Nuclear magnetic resonance (NMR) was performed and the symptoms were caused by associated mediastinal and retroperitoneal paragangliomas. The tumors were surgically removed. In a 3-year follow-up period the patient had two recidivate lesions, one of them was surgically removed, and for the last one a 6- month follow-up was recommended, because urine cathecholamine level was not significantly elevated and blood pressure was normal. Conclusion. Clinical and imaging data of patients with extra adrenal paragangliomas are not specific. Many of them may be asymptomatic even when the lesion is large, but if tumor is functional, diagnosis may be easier. Patients should be initially evaluated by determining catecholamine level, followed by computerized tomography (CT) or NMR to locate the primary lesion. Since there are no definite microscopic criteria for the distinction between benign and malignant tumors, radical excision and prolonged follow-up is necessary.