Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that may develop from extra-adrenal chromaffin cells, and most occur in the organ of Zuckerkandl. Extra-adrenal paraganglioma of the prostate is extremely rare. We report a 53-year-old man with hypertension and lower urinary tract symptoms, who was initially diagnosed with benign prostate hyperplasia. Computed tomography (CT) showed a large heterogenously enhancing mass in the prostate, imprinting the right distal ureter and urinary bladder. Before surgical intervention, CT-guided biopsy of the prostatic mass was performed and the result of histologic examination confirmed extra-adrenal paraganglioma. He underwent radical prostatectomy, partial cystectomy and right ureteroneocystostomy. The patient recovered and his blood pressure returned within normal range after surgical removal of the prostate tumour. In this article, we stress that the rarity of prostatic paraganglioma, preoperative localization and imaging-guided biopsy were useful in determining the surgical strategy.