Four neonates with the uncommon Cantrell's pentalogy seen in our hospital within a 6-month period without any apparent factor in common are described, with a review of the world literature. The first case was diagnosed prenatally at 15 weeks' gestation, the earliest intrauterine finding in the literature. The occurrence of imperforate anus with the syndrome, observed in one of the cases, has not been previously reported. Two were operated upon and only one of the four survived. This high mortality was confirmed by similar cases reported by other authors.