Arterio-venous malformation (AVM) is a congenital vascular malformation that is neither a venous malformation nor a haemangioma. An AVM is a potentially life-threatening and limb-threatening lesion, especially the 'fistulous' truncular form due to its unique embryological and haemodynamic characteristics. AVM treatment requires an early aggressive approach, one that is careful and based on a thorough assessment of the risks and benefits associated with the treatment plan. A successful treatment strategy requires an accurate assessment of the AVM taking into account the extent, severity and progression of the lesion. This is critical in order to minimize the morbidity associated with the currently available therapies. A multidisiciplinary approach that integrates endovascular and surgical therapy can substantially improve the treatment results seen in patients with AVMs. Preliminary treatment of a fistulous AVM with coil embolization is essential in order to minimize associated morbidity and to alter the lesion haemodynamics from a high-flow lesion to a low-flow lesion that is more amenable to subsequent, definitive management with ethanol or NBCA glue embolo/sclerotherapy.