Pulmonary arterial hypertension (PH) has a high prevalence in chronic kidney disease (CKD) patients, especially those undergoing kidney transplantation (KT). We aimed to systematically review and calculate the pooled effect size of the literature evaluating the association between pre-existing PH documented by transthoracic echocardiography (TTE) or invasively and adverse outcomes following KT. The primary composite outcome extracted from the included studies was represented by the mortality from any cause following KT and delayed graft function (DGF), graft dysfunction, or graft failure. The secondary outcomes were represented by individual components of the primary composite outcome. Twelve studies meeting the inclusion criteria were selected. The main finding is that pre-existing PH was associated with increased mortality and a higher rate of DGF, kidney graft dysfunction, or failure in KT recipients. The effect remained significant for all outcomes irrespective of PH evaluation, invasively or using TTE. Consequently, patients with PH defined only by TTE were at higher risk of death, DGF, or graft failure. Our findings support the routine assessment of PH in patients on the KT waitlist. PH might represent an extensively available and valuable tool for risk stratification in KT patients. These data should be confirmed in large prospective clinical trials.