We report an unusual case of tumour synchronism combining an extra-adrenal pheochromocytoma and a renal cell carcinoma in a 48-year-old male. The most usual forms of presentation are described in accordance with their clinical significance. We emphasize the diagnostic importance of specific biochemical studies and the accurate anatomical localization of extra-adrenal chromaffin tissue by magnetic resonance imaging (MRI) and scintigraphy with 131 Iodine-methyliodobenzylguanidine (131I-MIBG). Surgical excision was the treatment of choice after the institution of adequate preoperative cardiovascular management. Pertinent features of the natural history of these tumours are described in accordance with their different embryological origin.