BackgroundPulmonary stenosis (PS) is a congenital heart diseases (CHDs) with a spectrum of stenosis. Monochorionic (MC) twins are at increased risk of CHDs, especially acquired CHDs in twin-twin transfusion syndrome (TTTS). PS/Pulmonary atresia (PA) is a rare coincidence with TTTS. MC twin pregnancies have increased in last decades due to increasing in maternal age and extensive use of assisted reproductive technologies. Therefore, attention to this group is important for heart abnormalities, especially in twins with TTTS. Multiple cardiac abnormalities in MC twins with TTTS are to be expected due to cardiac hemodynamic changes and may be eliminated by Fetoscopic laser photocoagulation treatment. Prenatal diagnosis of PS is necessary given the importance of treatment after birth.Case presentationWe here present a case of coexistence of TTTS with PS in a growth restricted recipient twin who successfully treated with balloon pulmonary valvuloplasty in neonatal period. Also, we detected infundibular PS after valvuloplasty that treated with medical therapy (propranolol).ConclusionsIt is important to detect acquired cardiac abnormalities in MC twins with TTTS, and follow them up after birth to determine the need of intervention in neonatal period.