BACKGROUND: In Japan, transcatheter aortic valve replacement (TAVR) with self-expanding valves (SEV) was approved for patients with severe aortic stenosis (AS) and on chronic maintenance dialysis in May 2023. This study assessed the safety and efficacy of TAVR with SEVs in this patient population. METHODS: This prospective, non-randomized, single-center study evaluated the safety and effectiveness of TAVR using a supra-annular SEV in Japanese patients with severe AS on chronic maintenance dialysis. The primary endpoint of all-cause mortality or disabling stroke and several secondary endpoints were assessed at 12 months. RESULTS: Ten patients underwent TAVR using the Evolut platform (Medtronic, Minneapolis, MN, USA) between June 2020 and August 2022. Mean patient age was 80.0 ± 5.5 years and 70 % were male. Mean Society of Thoracic Surgeons Predicted Risk of Mortality score was 7.4 ± 2.2 %. At 1 year, the primary endpoint occurred in two patients (20 %), and there were no strokes, aortic valve reinterventions, or new permanent pacemaker implantations. Valve hemodynamics improved postprocedure and were maintained in all patients reaching 12-month follow-up (mean effective orifice area 2.1 ± 0.4 cm CONCLUSIONS: Japanese dialysis patients with severe AS who underwent TAVR using SEVs experienced favorable clinical and hemodynamic outcomes at 1-year follow-up. Further investigations are needed to assess long-term outcomes in this patient population.