Aorto-right ventricular fistula is a rare condition that communicates the ascending aorta to the right ventricle. This case describes a 31-year-old female attended at the outpatient clinic with a complain of dyspnea and edema in the lower limbs lasting about 2 months. Physical evaluation showed a holosystolic heart murmur on cardiac auscultation. Echocardiography and cardiac catheterization revealed a communication between the right coronary sinus to the right ventricular outflow tract. The patient underwent cardiac surgical treatment to correct the defect, in which fistula tissue was resected and the orifice was closed with bovine pericardium. The patient was discharged at the fifht day after procedure, and a postsurgical echocardiography did not show communication between the right sinus of valsalva to the right ventricle anymore.