Pheochromocytoma in a patient with end-stage renal disease is considered rare. A 40-year-old man who had undergone renal transplantation in childhood and had been on hemodialysis (HD) for the last 6 years suddenly developed paroxysmal palpitations and hypertension. His plasma catecholamine (CA) level was increased and a right adrenal mass was found on magnetic resonance imaging. He was diagnosed with pheochromocytoma, and right adrenalectomy was conducted after pretreatment with CA blockade and volume expansion. The surgery was conducted safely, his symptoms resolved, and his plasma CA level decreased to the normal range. Since paroxysmal hypertension is a common symptom in patients with HD, careful attention is needed to diagnose pheochromocytoma.