Pulmonary hypertension (PH) is a progressive, fatal pulmonary circulatory disease that is characterized by elevated pulmonary arterial pressure and secondary right ventricular failure. PH has been reported to be highly prevalent in patients with chronic kidney disease, and especially among end-stage renal disease patients undergoing haemodialysis. However, only few data are available on drug dosage adjustment to renal function for those drugs that are commonly used in the treatment of PH. We reviewed the literature and gathered information, although sparse, to propose guidelines for using these drugs in renal insufficiency patients.