Autologous arteriovenous fistula (AVF) is the preferable vascular access method for hemodialysis (HD), being a critical route for end-stage renal disease (ESRD) patients to maintain their lives. AVF dysfunction increases the hospitalization rate and mortality of ESRD patients. The mechanism of AVF dysfunction has not been fully elucidated, but insufficient vascular remodeling and neointimal hyperplasia have been considered as the main causes of non-thrombotic vascular stenosis. Toll-like receptor 4 (TLR4), an important member in the toll-like receptor family, mediates the proliferation and migration of vascular smooth muscle cells (VSMCs) and deposition of extracellular matrix in AVF wall, leading to intimal hyperplasia and AVF stenosis. Comprehension of TLR4 in the development of AVF dysfunction is closely related to the accurate diagnosis and treatment of AVF dysfunction and maintenance of long-term patency of AVF. Here we summarize the recent progresses in AVF stenosis, its influencing factors, TLR4, and the role of TLR4/NF-κB signal pathway in the pathogenesis of AVF dysfunction.