Paraganglioma of the urinary bladder is extremely rare. In this report of a young man, hypertensive crisis and ventricular arrhythmia was provoked during cystoscopic evaluation of a bladder mass. A diagnosis of pheochromocytoma was considered following detection of high serum and urinary catecholamine levels. A preoperative meta-iodobenzylguanidine scan was, however, negative. The bladder mass was surgically removed following initiation of antihypertensive therapy. Pathological confirmation of extraadrenal pheochromocytoma was established. During a serial follow-up, serum and urine catecholamine levels were persistently elevated. This was explained by abnormalities on fluorodeoxyglucose positron emission tomography scan, which were considered to represent a metastatic malignant neuroendocrine tumour. The patient is on palliative chemotherapy for malignant paraganglioma. This case highlights variable presentation of pheochromocytoma, importance of having a high index of clinical suspicion for early recognition and prompt management and serious adverse consequence of a delayed diagnosis.