The pentalogy of Cantrell (POC) is a rare syndrome with an incidence of 5.5 per 1 million live births with an unknown etiology. POC includes deficiency of the anterior diaphragm, defect in the supraumbilical abdominal wall in the midline and in the diaphragmatic pericardium, and also of the lower sternum and intracardiac abnormality. It has been suggested that the risk of birth defects are slightly increased in infants conceived by assisted reproductive technologies. Here, we reported the prenatal diagnosis of complete POC in an IVF/ICSI co-twin in the first trimester using two-dimensional ultrasound.