Renal failure and infectious disease are strongly associated with morbidity and mortality in patients with severe generalized recessive dystrophic epidermolysis bullosa (RDEB-sev gen). However, it is reportedly difficult to introduce hemodialysis with an arteriovenous fistula (AVF). We encountered a 32-year-old man with RDEB-sev gen in whom hemodialysis with a native AVF was introduced that favorably affected his long-term survival. This patient eventually died because of cachexia related to the recurrence of cutaneous squamous cell carcinoma 51 months after hemodialysis introduction. We believe that in this patient, the frequency of vascular access troubles related to infection or reduction of blood flow was probably low as a result of hemodialysis with his native AVF. Thus, it seems likely that patients with RDEB-sev gen with end stage kidney disease who are on hemodialysis can be successfully managed with a native AVF.