Pulmonary hypertension (PH) occurs in end-stage renal disease (ESRD) patients on long-term haemodialysis (HD) using an arterio-venous (A-V) access and can be attenuated by either kidney transplantation per se or surgical fistula ligation/revision. We report an exceptional case with severe PH after kidney transplantation due to ESRD and prior chronic intermittent HD via a patent A-V fistula. Gold-standard right heart catheterization findings have-for the first time-proven that following surgical shunt ligation of the A-V fistula, haemodynamics normalized completely in this patient.