Congenital diaphragmatic hernia (CDH) is associated with a variety of cardiac anomalies. However, its association with hypoplastic left heart syndrome (HLHS) is rare. We treated a female newborn with CDH, HLHS, and omphalocele. The operation for omphalocele and the diaphragmatic defect was successful, although the patient died of cardiac failure after Norwood's operation for HLHS. To our knowledge, this is the first reported case with a combination of these three major anomalies: CDH, HLHS, and omphalocele.