The Kidney Dialysis Outcomes Quality Initiative guidelines and the Fistula First Initiative for vascular access have had a measurable impact on the incidence and prevalence of the type of access used to deliver hemodialysis in the United States. There has been a yearly increase in the placement of arteriovenous fistulas and an exponential increase in endovascular treatment of failing and immature fistulas. Undoubtedly, the predominant cause of fistula malfunction is stenosis within the access system. The stenotic lesions can occur anywhere within the access system from the arteries to the outflow and central veins. One of the relatively common sites for stenosis in patients with brachiocephalic fistulas is the cephalic arch region. While access stenoses at many other sites have been successfully treated with percutaneous balloon angioplasty, the results of this approach in the management of cephalic arch stenosis (CAS) have been rather disappointing. This has been in part due to multiple factors including the resistant nature of the stenosis, the development of early restenosis, as well as poor patency and high vein rupture rates. This article discusses the anatomy, postulated etiology and percutaneous interventions for the treatment of CAS. In addition, the report highlights surgical alternatives to the management of stenosis in this segment of the cephalic vein.