Introduction: Fabry disease (FD) also known as Anderson Fabry disease is a rare disorder linked to the x chromosome, which produces mutations in the coding of the GLA gene involved in the production of the enzyme a-galactosidase A, whose complete or partial deficiency leads to the intracellular accumulation of globotriaosylceramide and glycosphingolipids. Clinical case: We present the case of a 39 year old female patient admitted to hospital with a diagnosis of terminal chronic kidney disease of 8 years of evolution as a possible cause of nephropathy, Fabry disease diagnosed in a patient, after detailed studies, kidney transplantation is considered for improvement of your lifestyle. discussion: Patients with Fabry disease should be considered as high risk surgical and anesthetic should have a strict assessment and evaluation of cardiovascular and respiratory function, to anticipate the complications associated with reperfusion of the transplanted organ. Conclusion: The use of balanced or intravenous modality has been described among the anesthetic possibilities without reaching a consensus so far, however the two modalities can be used and their analgesic management can be performed with plexus blocks or regional anesthesia. rESUMENIntroducción: La enfermedad de Fabry (FD) también conocida como enfermedad de Anderson Fabry es un trastorno raro ligado al cromosoma x, que produce mutaciones en la codificación del gen GLA partícipe en la producción de la enzima a-galactosidasa A, cuya deficiencia completa o parcial conduce a la acumulación intracelular de globotriaosilceramida y glicosfingolípidos.

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El personal de salud actualmente se enfrenta a múltiples desafíos en su práctica diaria, uno de ellos, es el manejo apropiado de anticoagulación de quienes por distintas causas demandan este tratamiento. Esta revisión bibliográfica ofrece pautas, especialmente para anestesiólogos, sobre el adecuado empleo de anticoagulantes y antiagregantes en pacientes que requieran anestesia/analgesia neuroaxial, bloqueos de plexo, bloqueos periféricos y catéteres; para evitar/prevenir complicaciones potencialmente devastadoras que el sangrado en el neuroaxis o sitios no compresibles  podría ocasionar. Se ha basado en las actuales recomendaciones de ASRA 2018.