Headache as a chief complaint is rare in the paediatric emergency room. Actually, very seldom cases secondary to life threatening conditions as non-traumatic subarachnoid haemorrhage have been reported. A child with severe headache and nuchal rigidity and no other abnormalities on the physical examination is reported. Magnetic resonance angiography and cerebral angiography disclosed a ventricular arteriovenous malformation in the choroid plexus, supplied by the anterior choroidal artery, classified according to Spetzler grading system as grade 3 (deep venous drainage: 1; eloquence area: 0 and size: 2). The differences in the clinical presentations of the central nervous system arteriovenous malformation between children and adults are discussed.