OBJECTIVES: To perform a systematic review (SR) of the efficacy of pharmacological treatment of Pulmonary Arterial Hypertension comparing Riociguat with other available medications or with placebo. METHODS: Following the steps described in the PRISMA guideline, a search for randomized controlled clinical trials was conducted in which Riociguat was used alone or in combination with other therapies, in databases MEDLINE, LILACS, Web of Science, Science Direct, Cochrane Library Wiley and in the gray literature (Google Scholar, Capes Bank of Theses and Clinical Trials). EndNote and Mendeley were used as reference managers. Outcomes analyzed were: death, 6-minutes walking distance (6MWD), WHO functional class (improvement, stabilization or worsening), hemodynamic variables (pulmonary vascular resistance, cardiac index, pulmonary-artery pressure), clinical worsening, hospitalization and quality of life. RESULTS: