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Autosomal dominant renal polycystic kidney disease (ADPKD) is one of the major causes for the development of chronic renal failure and renal transplantation. In 85.7% of the cases ADPKD is associated with hepatic polycystosis that could worsen the prognosis of the patient. We report a case of successful renal transplantation from related living donor in a patient with autosomal dominant polycystic disease with renal and hepatic involvement. Renal transplantation after good evaluation of the hepatic involvement is a safe renal replacement therapy in chronic renal failure patients. Due to a significantly high risk of postoperative infections after the transplantation close monitoring is recommended as well as prophylactic hepatitis A and B vaccination.

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