This syndrome was first introduced in 1983 when a nonconsanguineous couple had three daughters with similar lethal, multiple congenital anomalies. The family history was negative, and both parents had normal karyotypes. The firstborn had an omphalocele, posterior cleft palate, and uterus bicornis. Her birth weight was 2500 g. The second child had an omphalocele along with uvula duplex, and hydrocephalus internus with a birth weight of 2800 g. The last daughter’s birth weight was 3000 g, and she had an omphalocele and cleft palate. She was born at the 36th gestational week, and her karyotype was 46, XX (Czeizel 1983).