Background: Emerging data indicate that exercise training is beneficial in patients with pulmonary hypertension (PH). Methods: We performed a systematic review (Medline, EMBASE, LILACS) to identify RCT and observational studies reporting efficacy and safety of exercise training in patients with PH. Two of the authors independently selected studies and assessed methodological quality using GRADE approach. Primary outcomes included change in 6 min walking distance (6MWD), quality of life, cardiopulmonary exercise test parameters and safety. Results: We included 3 RTC and 8 cohorts (7 prospective), all with acceptable to high quality. RCTs included 77 patients (mean age 53 years, 88% women, NYHA class II-IV, HAP 45%, CTEHP 10%, monotherapy 29%). The cohorts included 1082 patients (mean age 45 years, 70% women, NYHA class II-IV, all Nice groups). For exercise training, all RCT and 7 observational studies showed statistically and clinically significant improvement in 6MWD; 8 studies evaluated quality of life, showing improvement in certain domains of SF36 score, without change in SGRQ score; 4 studies reported statistically significant changes in VO2máx postexcercise. The most frequent adverse events were respiratory infection, gastrointestinal symptoms and syncope/presyncope; no deaths related to excercise training were reported. Conclusions: Current evidence concerning the potential benefits and risks of exercise in patients with PH is encouraging, as it increases VO2máx post-excercise and improves 6MWT as well as certain aspects of quality of life. RCTs combining excercise training and self-education are needed to evaluate the benefits of pulmonary rehabilitation in PH.