Spontaneous arterio-venous fistulae of the vertebral artery are rare. These lesions mainly affect the upper cervical area, and are usually asymptomatic, or may present as small, often pulsatile, cervical masses with vascular murmurs. The authors report on two cases in which the presumptive diagnosis, suggested by venous digital subtraction angiography, was then confirmed by selective angiography. In both cases an intravascular approach with detachable balloons and particulate substances was carried out, with good anatomical and functional results. Problems related to diagnosis, pathophysiology of symptoms, indications for treatment and embolization techniques are discussed.