We report an 18-year-old man who presented with a sudden onset of headache followed by left hemianopia. A brain CT scan showed intracerebral haemorrhage in the left frontoparietal area, but a cerebral angiogram and MRI revealed no vascular anomaly. The patient was managed conservatively and his headache and visual loss improved over time. Hypertension in the form of paroxysmal attacks led us to suspect phaeochromocytoma. Subsequently, the patient was diagnosed with an extra-adrenal phaeochromocytoma in the left para-aortic area following endocrinological evaluation, abdominal CT scan and (131)I-meta-iodobenzylguanidine (MIBG) scintigraphy. The patient presented here illustrates the importance of a careful search for a remediable cause of hypertension in children and young adults with spontaneous intracerebral haemorrhage.