This publication aims to provide an overview of the clinical course, treatment andperinatal outcome of twin pregnancies discordant for intrapericardial teratoma.Following PRISMA and Cochrane guidelines for systematic reviews, we selected allpublications that published on prenatally diagnosed twin pregnancies discordantfor intrapericardial teratoma. Seven publications were found, all of which were casereports and were included in the final analysis, plus a recent case presented in thispublication. To date, 8 cases of twin pregnancies discordant for intrapericardialteratoma have been reported. All (8/8) had severe pericardial effusion, 5 (62.5%)developed hydrops and 2/8 (25%) polyhydramnios. Five (62.5%) cases were treatedwith pericardiocentesis and 2 (25%) underwent concomitant amniodrainage becauseof polyhydramnios. All cases were delivered by cesarean section with a mediangestational ag...