We report the prenatal diagnosis of 2 cases of Pentalogy of Cantrell in the first trimester. In case 1, sonographic evaluation revealed ectopia cordis, omphalocele, and cystic hygroma at 10 weeks' gestation. In case 2, sonographic assessment during the first trimester detected ectopia cordis and omphalocele at 11 weeks' gestation. In both cases, the patient opted for elective pregnancy termination, and Pentalogy of Cantrell was confirmed in 2 male fetuses. We discuss the role of Doppler imaging and 3-dimensional sonography as complementary methods to conventional sonographic assessment of abdominal wall defects at early pregnancy.