The Pentalogy of Cantrell is a rare congenital anomaly characterized by defects in the closing of the supraumbilical abdominal wall, in the anterior portion of the diaphragm, in the diaphragmatic pericardium, ectopia cordis, and intracardiac defects. It may be associated to other anomalies determining the variants of this syndrome. The prenatal ultrasound diagnosis is extremely important for detecting structural malformations and for postnatal surgical planning, when possible. We present a case of Pentalogy of Cantrell diagnosed in the 34th week of gestation, showing the main findings by two-dimensional ultrasound and emphasizing the importance of three-dimensional ultrasound on the assessment of structural malformations and on the better understanding of the origins of the pathology.