OBJECTIVE To describe a case of bladder paraganglioma with an uncommon form of presentation. The clinical features and diagnostic aspects of this condition are briefly reviewed. RESULTS Herein we describe the clinical history of a young male patient that had been misdiagnosed as having a ureterocele causing ureterohydronephrosis and hypertension, and had undergone nephrectomy. Pathological analysis of the surgical specimen revealed the underlying cause of the patients's condition. CONCLUSIONS Pheochromocytoma is a rare disease entity whose most common form of presentation is hematuria or that which results from catecholamine produced by the tumor. Lesions located close to the ureteral meatus may cause obstruction. If this condition is not suspected and the findings of diagnostic imaging are inadequately interpreted, its diagnosis and treatment may be delayed, thereby increasing the surgical risk due to its secretory nature.