ABSTRACT
The modern concept of some embryological structures found as dispersed cells at the retroperitoneal region, is to be considered as a great group of structures with similar embryological origin that can be divided in various anatomical and functional group. A case of a malignant extra-adrenal retroperitoneal paraganglioma is described in a 14 year-old man who presented an episode of hematuria following a history of trauma. A computed tomography was performed and showed a solid mass in lower pole of the left kidney. A magnetic resonance imaging examination was later indicated. Because of the persistence of the mass, a surgical exploration was performed and the tumor was removed. The pathological examination demonstrated a malignant paraganglioma. These tumors are rare and difficult to diagnose pre-operatively. The prognosis is poor and the best results reported are around 3 years survival