Arteriovenous access-related pulmonary arterial hypertension is a previously under-diagnosed complication in hemodialysis patients, and should be kept in mind when hemodialysis patients using surgically created arteriovenous access encounter unexplained dyspnea. It was found that successful renal transplantation, peritoneal dialysis and hemodialysis with a permanent dialysis catheter can reduce pulmonary arterial pressure in this group of patients. The patient reported herein had severe pulmonary arterial hypertension but insisted on using an arteriovenous fistula for hemodialysis. Her pulmonary arterial pressure, dyspnea and oxygenation improved after ligation of the original high-flow arteriovenous fistula and creation of a low-flow alternative. Although the pulmonary arterial pressure did not decrease to a normal range, the patient's clinical symptoms and quality of life clearly improved. This case may provide new insight into the management of pulmonary arterial hypertension in this group of patients.