Pentalogy of Cantrell (PC) is a rare congenital anomaly involving defects in the anterior diaphragm, supraumbilical abdominal wall, diaphragmatic pericardium, and lower sternum, and other congenital intracardiac abnormalities. Here, we report the case of a newborn infant who was born at 32 weeks of gestation and had all 5 features of PC, in addition to absent kidneys and a deformed left hand. Medical intervention would not be able to save the patient, so we allowed her to die in peace. We discuss here the etiology, prenatal diagnosis, and severity of and the mortality associated with this condition. To our knowledge, this was the first reported case of PC in Saudi Arabia.