We report the radiological features, including Doppler sonography and magnetic resonance angiography (MRA) findings, of a nongalenic arteriovenous fistula diagnosed in the neonatal period. Hypertensive hydrocephalus developed in infancy. Emergent percutaneous transarterial embolization with n-butyl-2-cyanoacrylate was successfully performed and lead to clinical improvement. MRA allowed a noninvasive follow-up.