Abstract: Fabry disease (FD) is an X-linked lysosomal storage disorder, with the kidney as the major affected organ. Approximately 50% of male patients may progress to end-stage renal disease. Kidney transplantation serves as an effective therapeutic approach for FD-related end-stage renal disease; however, transplantation only corrects uremia and cannot reverse the deposition of globotriaosylceramide (Gb₃) in extra-renal organs such as the heart and brain. The leading cause of death in post-transplant patients has shifted from uremia to cardiovascular and cerebrovascular events, with prognostic characteristics significantly different from those of conventional kidney transplant recipients. At present, the perioperative management of kidney transplantation for FD mostly adopts routine protocols, and individualized strategies targeting systemic lesions of FD are lacking. The clinical value of enzyme replacement therapy after transplantation and its interaction with immunosuppressive agents remain to be clarified. This article systematically reviews the perioperative management and long-term follow-up strategies of kidney transplantation in patients with FD-related end-stage renal disease, elaborating on pre-transplant risk stratification, key points of perioperative management, combined application of enzyme replacement therapy and immunosuppression, and the long-term multisystem collaborative management model. It aims to establish a framework of individualized immunosuppression and multisystem comprehensive management, reduce the risk of cardiovascular and cerebrovascular complications, and improve the long-term survival and quality of life of Fabry disease patients after kidney transplantation.