Central vein stenosis is commonly associated with placement of central venous catheters and devices. Central vein stenosis can jeopardize the future of arteriovenous fistula and arteriovenous graft in the ipsilateral extremity. Occurrence of central vein stenosis in association with indwelling intravascular devices including short-term, small-diameter catheters such as peripherally inserted central catheters, long-term hemodialysis catheters, as well as pacemaker wires, has been recognized for over two decades. Placement of multiple catheters, longer duration, location in subclavian vein, and placement on the left-hand side of neck seem to predispose to the development of central vein stenosis. Endothelial injury with subsequent changes in the vessel wall results in development of microthrombi, smooth muscle proliferation, and central vein stenosis. Central vein stenosis is often asymptomatic in nondialysis patients, but can result in edema of ipsilateral extremity and breast when challenged by increased flow from an arteriovenous fistula or arteriovenous graft. Bilateral central vein stenosis or superior vena cava stenosis can produce a clinical picture of superior vena cava syndrome, associated with engorgement of face and neck. Endovascular interventions are the mainstay of management of central vein stenosis. Percutaneous angioplasty and stent placement for elastic and recurring lesions can restore the functionality of the vascular access, at least temporarily. Frequent or multiple interventions are usually required. In recalcitrant cases, surgical bypass of the obstruction is an option. In resistant cases with severe symptoms, occlusion of the functioning vascular access will usually provide relief of symptoms. Further study of mechanisms of development of central vein stenosis and search for a targeted therapy is likely to lead to better ways of managing central vein stenosis. Prevention of central vein stenosis is the key to avoid access failure and other complications from central vein stenosis and relies upon avoidance of central vein stenosis placement and timely placement of arteriovenous fistula in prospective dialysis patient.