Unilateral agenesis of the diaphragm with bilateral renal agenesis is an extremely rare lethal developmental anomaly. A detailed evaluation of fetal organs during nuchal translucency scan is required to diagnose this type of developmental anomaly. Detecting lethal developmental abnormality at an early gestational period (11–14 weeks) will be invaluable in facilitating the decision to terminate the pregnancy. Termination in the first trimester reduces psychological stress to the parents. We present a rare case of combined developmental anomaly which has never been reported in the literature.