A high-flow access has been defined as one with a flow >1-1.5 l/minute or as one where the access flow is >20% of the cardiac output. Although it may be asymptomatic, it can be associated with cardiopulmonary complications, aneurysms, central vein stenosis and the distal hypoperfusion ischemic syndrome in some patients. The decision for surgical intervention should be made after careful medical optimization especially in patients with high output cardiopulmonary compromise. In this review, we present a summary of current knowledge about the pathophysiology of a high-flow fistula, followed by a concise, comprehensive synopsis of current medical and surgical therapy.