RESUMO
The pentalogy of Cantrell is a rare congenital syndrome characterized by deficiency of the anterior diaphragm and defects of abdominal wall, the pericardium, the lower sternum, as well as congenital intracardiac abnormalities. It has usually a poor prognosis, but most cases have had incomplete variants of this syndrome, so it is important to make a prenatal diagnosis to determine the size of the wall defect and to establish a multidisciplinary management. Less than 90 cases have been reported in the world literature. There are no casuistic or even treatment criteria in Latin America. A case of a newborn in whom was suspected this pentalogy associated to bilateral cleft lip by an ultrasound examination at 25 week of gestation is described. We also comment on diagnostic aspects, as well as anatomopathological, therapeutic, and prognostic characteristics.