Prenatal echocardiographic diagnosis of heart defects is important because it permits counseling of the parents with regard to prognosis and treatment options and prepares the medical team for the treatment postnatally. A male infant with absent pulmonary valve syndrome diagnosed prenatally at 22 weeks' gestation is reported. This congenital anomaly is characterized by absent or rudimentary pulmonary valve cusps, conoventricular septal defect, and massive dilatation of the pulmonary arteries. Soon after delivery the infant developed cyanosis and respiratory distress. The infant was placed in prone position for the relief of bronchial compression and nasal continuous positive airway pressure was (CPAP) started. Although the clinical status of the infant improved after supportive treatment, he deteriorated acutely and died at the age of five days. Fetal diagnosis remains an integral part of successful management of children with heart disease. Despite the potential benefits of prenatal diagnosis, it is hard to show significant improvement in mortality, especially in severely affected cases.