CASE PRESENTATION: The patient was a 24-years-old woman with three-years history of tachycardia, chest pain, syncope and secondary convulsive syndrome. At physical examination: mild cyanosis, auscultation with P2 reinforcement but no murmurs, crackles or wheezing, and evidence of hepatomegaly and inferior limb edema. Chest Rx showed prominent pulmonary artery without lung infiltrates. Echocardiogram with PASP 124 mmHg, severe dilatation of RV, tricuspid insufficiency and small-size LV. Chest CT (-) for pulmonary embolism without lung infiltrates. Spirometry and CO diffusion were normal. Right heart catheterization reported EFLV of 65%, PAP 134 mm Hg, severe dilatation of RV, no intracardiac shunt and normal wedge pressure. Combined treatment was initiated but multiple admissions occurred due to decompensation with RHF. On 2009, SAB was performed followed by adequate symptoms’ control. The patient was discharged with triple therapy, Bosentan, Sildenafil and IV Epo, since 2010, with clinical relief and good quality of life to the date.