Maaswinkel-Mooij and Stokvis-Brantsma [1992], Meacham et al.[1991], and Sorgo et al.[1991] independently reported four patients with a constellation of specific genital, pulmonary and cardiac malformations, normal karyotype, and negative family history of similar defects, consanguinity or known exposure to teratogens. The patients were term infants of normal size with hypoplastic right lungs, abnormalities of the ipsilateral diaphragm, anomalous pulmonary venous return, left ventricular hypoplasia, and shift of the heart to the right hemithorax (dextroposition). All had a male 46, XY karyotype, hypo-or agonadism and undervirilization of external male genitalia with retention of Müllerian structures. Given the unknown etiology, Kennerknecht et al.[1993] proposed the term PAGOD syndrome, an acronym referring to pulmonary and pulmonary artery hypoplasia, agonadism, omphalocele, diaphragmatic defect, and dextrocardia. Similar abnormalities, called vitamin A-deficiency syndrome, were described in embryos from vitamin A-deficient (VAD) pregnant rats [Clagett-Dame and Deluca, 2002]. These defects overlap with the features observed in children with PAGOD syndrome and