A rare case of complex cerebral arteriovenous fistula in an infant is reported. An 8-month-old boy first presented with a syndrome of increased intracranial pressure. Neuroradiological assessment showed a direct intracerebral arteriovenous shunt with marked venous engorgement. No hemorrhage was evident. Direct surgical treatment was decided. Clinical and radioanatomical cure was achieved. The main angiographic and pathophysiologic features of this unusual entity, as well as the available therapeutic options, are reviewed and discussed.