Arteriovenous hemodialysis fistulas (AVFs) serve as a lifeline for many individuals with end-stage renal failure. A common cause of AVF failure is cephalic arch stenosis. Its high prevalence compounded with its resistance to treatment makes cephalic arch stenosis important to understand. Proposed etiologies include altered flow in a fistulized cephalic vein, external compression by fascia, the unique morphology of the cephalic arch, large number of valves in the cephalic outflow tract and biochemical changes that accompany renal failure. Management options are also in debate and include angioplasty, cutting balloon angioplasty, bare metal stents, stent grafts and surgical techniques including flow reduction with minimally invasive banding as well as more invasive venovenostomy with transposition surgeries for refractory cases. In this review, the evidence for the clinical relevance of cephalic arch stenosis, its etiology and management are summarized.