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We report a 18-year-old man, who has been taking antihypertensive medication for 1month in a local clinic, presented with a sudden onset headache followed by left blindness. He experienced palpitation and chest discomfort during physical exertion since 2years before admission, but unfortunately has been ignored. Brain CT showed intracerebral hemorrhage in the left temporoparietal area, but cerebral angiogram and magnetic resonance image revealed no vascular anomaly. He was managed conservatively, and headache and visual loss were improved over time. Subsequently, on the evaluation of hypertension, he was diagnosed as having extra-adrenal pheochromocytoma on left paraaortic area from the results of endocrinological evaluations, abdominal CT scan, and 131I-MIBG scintigraphy.