We report about the prenatal diagnosis of pentalogy of Cantrell in the third trimester. In this case sonographic evaluation revealed mild form of ectopia cordis, severe omphalocele, small meningomyelocele, mild hydrocephalus, severe polyhydramnios and allantoic cyst of the umbilical cord. The pregnancy was terminated by a caesarean section at 35 weeks of pregnancy because of severe polyhydramnios. The neonate died shortly after delivery and the obduction confirmed the diagnosis of pentalogy of Cantrell. We discuss the reason of the late prenatal diagnosis in this case, the importance of early prenatal diagnosis and the options of pregnancy management.